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- Novel Substituted Heterocyclic and Heteroaryl Compounds for Treating Huntington's Disease [NCBI]ACS Med Chem Lett. 2021 Jul 20;12(8):1204-1205. doi: 10.1021/acsmedchemlett.1c00356. eCollection 2021 Aug 12.NO ABSTRACTPMID:34413943 | PMC:PMC8365614 | DOI:10.1021/acsmedchemlett.1c00356 ... read more
- Extracellular vesicles, stem cells and the role of miRNAs in neurodegeneration [NCBI]Curr Neuropharmacol. 2021 Aug 17. doi: 10.2174/1570159X19666210817150141. Online ahead of print.ABSTRACTThere are different modalities of intercellular communication governed by cellular homeostasis. In this review, we will explore one of these forms of communication called extracellular vesicles (EVs). These vesicles are released by all cells in the body and are heterogeneous ... read more
- Effects of Educative Materials on Doctors' Intention to Initiate Life-Saving Procedures After a Suicide Attempt: Randomised Controlled Trial [NCBI]Front Psychol. 2021 Aug 3;12:718084. doi: 10.3389/fpsyg.2021.718084. eCollection 2021.ABSTRACTIntroduction: The topic of euthanasia, assisted dying, and how to deal with death wishes has received strong public and media attention in many countries. Nevertheless, there is currently no research which has analysed if educative materials that favour or disfavour the initiation ... read more
- Rescue of BDNF Expression by the Thalamic Parafascicular Nucleus with Chronic Treatment with the mGluR2/3 Agonist LY379268 may Contribute to the LY379268 Rescue of Enkephalinergic Striatal Projection Neurons in R6/2 Huntington's Disease Mice [NCBI]Neurosci Lett. 2021 Aug 17:136180. doi: 10.1016/j.neulet.2021.136180. Online ahead of print.ABSTRACTWe have found that daily subcutaneous injection with a maximum tolerated dose of the mGluR2/3 agonist LY379268 (20 mg/kg) beginning at 4 weeks of age dramatically improves the motor, neuronal and neurochemical phenotype in R6/2 mice, a rapidly progressing transgenic ... read more
- 5,6,7 trihydroxy flavone armoured neurodegeneration caused by Quinolinic acid induced huntington's like disease in rat striatum – reinstating the level of brain neurotrophins with special reference to cognitive-socio behaviour, biochemical and histopathological aspects [NCBI]Neurosci Res. 2021 Aug 17:S0168-0102(21)00188-7. doi: 10.1016/j.neures.2021.08.003. Online ahead of print.ABSTRACTHuntington Disease (HD), a predominant Neurodegenerative Disorder which might be induced by endogenous neurotoxin called Quinolinic Acid (QA), an N-methyl-D aspartate receptor (NMDAR) agonist, the bilaterally intrastriatal administration (200 nm/2 µl of saline) offers rise to the toxic events like ... read more
- Fluc-EGFP reporter mice reveal differential alterations of neuronal proteostasis in aging and disease [NCBI]EMBO J. 2021 Aug 19:e107260. doi: 10.15252/embj.2020107260. Online ahead of print.ABSTRACTThe cellular protein quality control machinery is important for preventing protein misfolding and aggregation. Declining protein homeostasis (proteostasis) is believed to play a crucial role in age-related neurodegenerative disorders. However, how neuronal proteostasis capacity changes in different diseases is not ... read more
- In vivo Direct Conversion of Astrocytes to Neurons Maybe a Potential Alternative Strategy for Neurodegenerative Diseases [NCBI]Front Aging Neurosci. 2021 Aug 2;13:689276. doi: 10.3389/fnagi.2021.689276. eCollection 2021.ABSTRACTPartly because of extensions in lifespan, the incidence of neurodegenerative diseases is increasing, while there is no effective approach to slow or prevent neuronal degeneration. As we all know, neurons cannot self-regenerate and may not be replaced once being damaged or ... read more
- A Curious Case of Excessive Winking [NCBI]Mov Disord Clin Pract. 2021 May 29;8(6):947-949. doi: 10.1002/mdc3.13254. eCollection 2021 Aug.NO ABSTRACTPMID:34405103 | PMC:PMC8354074 | DOI:10.1002/mdc3.13254 ... read more
- FOXO and related transcription factors binding elements in the regulation of neurodegenerative disorders [NCBI]J Chem Neuroanat. 2021 Aug 13:102012. doi: 10.1016/j.jchemneu.2021.102012. Online ahead of print.ABSTRACTNeurodegenerative diseases, such as Alzheimer's disease, Parkinson's disease, Huntington's disease, amyotrophic lateral sclerosis, and others, are characterized by progressive loss of neuronal cells, which causes memory impairment and cognitive decline. Mounting evidence demonstrated the possible implications of diverse biological ... read more
- Significant Association Between Huntingtin Gene Mutation and Prevalence of Hopelessness, Depression and Anxiety Symptoms [NCBI]Acta Med Litu. 2021;28(1):77-85. doi: 10.15388/Amed.2020.28.1.4. Epub 2021 Jan 21.ABSTRACTSUMMARY BACKGROUND: In Huntington's disease psychiatric symptoms may manifest prior to motor dysfunction. Such symptoms negatively impact people's quality of life and can worsen the course of the primary disease. The aim of the present study was to assess and compare ... read more
- Metabolic pathways controlled by E3 ligases: an opportunity for therapeutic targeting [NCBI]Biol Aujourdhui. 2021;215(1-2):45-57. doi: 10.1051/jbio/2021006. Epub 2021 Aug 16.ABSTRACTSince its discovery, the Ubiquitin Proteasome System (UPS) has been recognized for its major role in controlling most of the cell's metabolic pathways. In addition to its essential role in the degradation of proteins, it is also involved in the addressing, signaling ... read more
- HDinHD: A Rich Data Portal for Huntington's Disease Research [NCBI]J Huntingtons Dis. 2021 Aug 8. doi: 10.3233/JHD-210491. Online ahead of print.ABSTRACTHDinHD (Huntington's Disease in High Definition; HDinHD.org) is an open online portal for the HD research community that presents a synthesized view of HD-related scientific data. Here, we present a broad overview of HDinHD and highlight the newly launched ... read more
- In silico Analysis of Transcriptomic Profiling and Affected Biological pathways in Multiple Sclerosis [BioRxiv_Neuroscience]Multiple sclerosis (MS) is a chronic autoimmune, inflammatory neurological disease that is widely associated with Grey and white matter degradation due to the demyelination of axons. Thus exposing the underlying causes of this condition can lead to a novel treatment approach for Multiple Sclerosis. The total RNA microarray processed data ... read more
- Mood and emotional disorders associated with parkinsonism, Huntington disease, and other movement disorders [NCBI]Handb Clin Neurol. 2021;183:175-196. doi: 10.1016/B978-0-12-822290-4.00015-3.ABSTRACTThis chapter provides a review of mood, emotional disorders, and emotion processing deficits associated with diseases that cause movement disorders, including Parkinson's disease, Lewy body dementia, multiple system atrophy, progressive supranuclear palsy, corticobasal degeneration, frontotemporal dementia with parkinsonism, Huntington's disease, essential tremor, dystonia, and tardive ... read more
- Disorders of facial emotional expression and comprehension [NCBI]Handb Clin Neurol. 2021;183:99-108. doi: 10.1016/B978-0-12-822290-4.00006-2.ABSTRACTOne of the most important means of communicating emotions is by facial expressions. About 30-40 years ago, several studies examined patients with right and left hemisphere strokes for deficits in expressing and comprehending emotional facial expressions. The participants with right- or left-hemispheric strokes attempted to ... read more
- RNA Toxicity and Perturbation of rRNA Processing in Spinocerebellar Ataxia Type 2 [NCBI]Mov Disord. 2021 Aug 14. doi: 10.1002/mds.28729. Online ahead of print.ABSTRACTBACKGROUND: Spinocerebellar ataxia type 2 (SCA2) is a neurodegenerative disease caused by expansion of a CAG repeat in Ataxin-2 (ATXN2) gene. The mutant ATXN2 protein with a polyglutamine tract is known to be toxic and contributes to the SCA2 pathogenesis.OBJECTIVE: ... read more
- Rescue of aberrant huntingtin palmitoylation ameliorates mutant huntingtin-induced toxicity [NCBI]Neurobiol Dis. 2021 Aug 11:105479. doi: 10.1016/j.nbd.2021.105479. Online ahead of print.ABSTRACTHuntington disease (HD) is a neurodegenerative disorder caused by a CAG expansion in the HTT gene that codes for an elongated polyglutamine tract in the huntingtin (HTT) protein. HTT is subject to multiple post-translational modifications (PTMs) that regulate its cellular ... read more
- HAP40 protein levels are huntingtin-dependent and decrease in Huntington disease [NCBI]Neurobiol Dis. 2021 Aug 11:105476. doi: 10.1016/j.nbd.2021.105476. Online ahead of print.ABSTRACTThe huntingtin-associated protein 40 (HAP40) is an abundant interactor of huntingtin (HTT). In complexes of these proteins, HAP40 tightly binds to HTT in a cleft formed by two larger domains rich in HEAT repeats, and a smaller bridge domain connecting ... read more
- Generation of striatal neurons from human induced pluripotent stem cells by controlling extrinsic signals with small molecules [NCBI]Stem Cell Res. 2021 Aug 4;55:102486. doi: 10.1016/j.scr.2021.102486. Online ahead of print.ABSTRACTHuman induced pluripotent stem cells (hiPSCs) are powerful tools for modeling human brain development and treating neurodegenerative diseases. Here we established a robust protocol with high scalability for generating striatal medium spiny neurons (MSNs) from hiPSCs using small molecules ... read more
- Health services utilization of Chinese patients with Huntington's disease: a cross-sectional study [NCBI]BMC Health Serv Res. 2021 Aug 12;21(1):806. doi: 10.1186/s12913-021-06826-1.ABSTRACTBACKGROUND: Huntington's disease (HD) is a hereditary disease which could have a large impact on patients' quality of life. As the neurodegenerative disorders progress, HD patients are expected to regularly take follow-up medical visits for proper treatment. This study aimed to analyze ... read more
- Natural compounds modulate the autophagy with potential implication of stroke [NCBI]Acta Pharm Sin B. 2021 Jul;11(7):1708-1720. doi: 10.1016/j.apsb.2020.10.018. Epub 2020 Oct 29.ABSTRACTStroke is considered a leading cause of mortality and neurological disability, which puts a huge burden on individuals and the community. To date, effective therapy for stroke has been limited by its complex pathological mechanisms. Autophagy refers to an ... read more
- Approach to Posture and Gait in Huntington's Disease [NCBI]Front Bioeng Biotechnol. 2021 Jul 27;9:668699. doi: 10.3389/fbioe.2021.668699. eCollection 2021.ABSTRACTDisturbances of gait occur in all stages of Huntington's disease (HD) including the premanifest and prodromal stages. Individuals with HD demonstrate the slower speed of gait, shorter stride length, and increased variability of gait parameters as compared to controls; cognitive disturbances ... read more
- Profile of the RNA in exosomes from astrocytes and microglia using deep sequencing: implications for neurodegeneration mechanisms [NCBI]Neural Regen Res. 2022 Mar;17(3):608-617. doi: 10.4103/1673-5374.320999.ABSTRACTGlial cells play an important role in signal transduction, energy metabolism, extracellular ion homeostasis and neuroprotection of the central nervous system. However, few studies have explained the potential effects of exosomes from glial cells on central nervous system health and disease. In this study, ... read more
- Investigating Crosstalk Among PTMs Provides Novel Insight Into the Structural Basis Underlying the Differential Effects of Nt17 PTMs on Mutant Httex1 Aggregation [NCBI]Front Mol Biosci. 2021 Jul 26;8:686086. doi: 10.3389/fmolb.2021.686086. eCollection 2021.ABSTRACTPost-translational modifications (PTMs) within the first 17 amino acids (Nt17) of the Huntingtin protein (Htt) have been shown to inhibit the aggregation and attenuate the toxicity of mutant Htt proteins in vitro and in various models of Huntington's disease. Here, we ... read more
- Cognition-oriented treatments and physical exercise on cognitive function in Huntington's disease: Protocol for systematic review [MedRxiv_Neurology]Introduction: Cognitive impairments are prevalent in Huntington's disease (HD), occurring many years prior to clinical diagnosis and are the most impactful on quality of life of patients. Cognitive interventions and exercise have been found to be efficacious in improving cognitive function in several clinical populations (e.g., older adults with mild ... read more
- Gut bacteria regulate the pathogenesis of Huntington's disease in Drosophila [BioRxiv_Neuroscience]Changes in the composition of gut microbiota are implicated in the pathogenesis of several neurodegenerative disorders. Here, we investigated whether gut bacteria affect the hallmarks of Huntington's disease (HD) in transgenic Drosophila melanogaster (fruit fly) models expressing human full-length or N-terminal fragments of mutant huntingtin (HTT) protein, here referred to ... read more
- Potential of genistein-induced autophagy in the treatment of neurodegenerative diseases [NCBI]Postepy Biochem. 2021 Apr 26;67(2):117-129. doi: 10.18388/pb.2021_380. Print 2021 Apr 30.ABSTRACTDevelopment of therapies for neurodegenerative diseases, disorders characterized by progressing loss of neurons, is a great challenge for current medicine. Searching for drugs for these diseases is being proceeded in many laboratories in the world. To date, several therapeutical strategies ... read more
- Juvenile Huntington's disease masquerading as progressive myoclonus epilepsy [NCBI]Epilepsy Behav Rep. 2021 Jul 15;16:100470. doi: 10.1016/j.ebr.2021.100470. eCollection 2021.ABSTRACTJuvenile Huntington's disease (JHD) has an onset before 20 years of age, and is characterized by behavioural issues, epilepsy, rigidity, bradykinesia and dystonia. It contributes to 0.5-5% of all Huntington disease (HD) cases. JHD demonstrates a more rapid progression and is ... read more
- Allele-specific knockdown of mutant HTT protein via editing at coding region SNP heterozygosities [NCBI]Hum Gene Ther. 2021 Aug 10. doi: 10.1089/hum.2020.323. Online ahead of print.ABSTRACTHuntington's disease (HD) is a devasting, autosomal dominant neurodegenerative disease caused by a trinucleotide repeat expansion in the HTT gene. Inactivation of the mutant allele by CRISPR-Cas9 based gene editing offers a possible therapeutic approach for this disease, but ... read more
- PLGA-Based Nanoparticles for Neuroprotective Drug Delivery in Neurodegenerative Diseases [NCBI]Pharmaceutics. 2021 Jul 8;13(7):1042. doi: 10.3390/pharmaceutics13071042.ABSTRACTTreatment of neurodegenerative diseases has become one of the most challenging topics of the last decades due to their prevalence and increasing societal cost. The crucial point of the non-invasive therapeutic strategy for neurological disorder treatment relies on the drugs' passage through the blood-brain barrier ... read more
- The experience of a sample of individuals in the United Kingdom living in the pre-manifest stage of Huntington's disease: An interpretative phenomenological analysis [NCBI]J Genet Couns. 2021 Aug 10. doi: 10.1002/jgc4.1497. Online ahead of print.ABSTRACTThis paper explores the experience of ten individuals living in the UK who were in the pre-manifest stage of Huntington's disease (HD), a genetic neurodegenerative condition. Data were gathered using semi-structured interviews and analyzed using interpretative phenomenological analysis. Three ... read more
- Role of YAP in early ectodermal specification anda Huntington's Disease model of human neurulation [BioRxiv_Developmental_Biology]The Hippo pathway, a highly conserved signaling cascade that functions as an integrator of molecular signals and biophysical states, ultimately impinges upon the transcription coactivator Yes-associated protein 1 (YAP). Hippo-YAP signaling has been shown to play key roles both at the early embryonic stages of implantation and gastrulation, and later ... read more
- Impaired Cerebrovascular Reactivity in Huntington's Disease [NCBI]Front Physiol. 2021 Jul 21;12:663898. doi: 10.3389/fphys.2021.663898. eCollection 2021.ABSTRACTThere is increasing evidence that impairments of cerebrovascular function and/or abnormalities of the cerebral vasculature might contribute to early neuronal cell loss in Huntington's disease (HD). Studies in both healthy individuals as well as in patients with other neurodegenerative disorders have used ... read more
- Pet Therapy as a Nonpharmacological Treatment Option for Neurological Disorders: A Review of the Literature [NCBI]Cureus. 2021 Jul 4;13(7):e16167. doi: 10.7759/cureus.16167. eCollection 2021 Jul.ABSTRACTAnimal therapy and ownership have been studied as a nonpharmacologic treatment option for cardiovascular and psychological disorders. Animal companionship is less studied in neurological disorders such as stroke, dementia, Parkinson's disease, multiple sclerosis, Huntington's disease, epilepsy, and acute brain injury. This review ... read more
- DNM3OS regulates GAPDH expression and influences the molecular pathogenesis of Huntington's disease [NCBI]J Cell Mol Med. 2021 Aug 8. doi: 10.1111/jcmm.16838. Online ahead of print.ABSTRACTEmerging studies have suggested that dysregulated long non-coding RNAs (lncRNAs) are associated with the pathogenesis of neurodegenerative diseases (NDD) including Huntington's disease (HD); however, the pathophysiological mechanism by which lncRNA dysregulation participates in HD remains to be elucidated. ... read more
- Subdural Hematoma as a Serious Complication of Huntington's Disease: An Observational Study [NCBI]J Huntingtons Dis. 2021 Aug 4. doi: 10.3233/JHD-210478. Online ahead of print.ABSTRACTBACKGROUND: Persons with Huntington's disease (HD) are at increased risk for subdural hematomas (SDH) because of underlying brain atrophy and increased frequency of falls and head trauma. SDH can cause serious disability, but there is little information about the ... read more
- Abnormal Spinal Cord Myelination due to Oligodendrocyte Dysfunction in a Model of Huntington's Disease [NCBI]J Huntingtons Dis. 2021 Aug 3. doi: 10.3233/JHD-210495. Online ahead of print.ABSTRACTBACKGROUND: The relative contribution of grey matter (GM) and white matter (WM) degeneration to the progressive brain atrophy in Huntington's disease (HD) has been well studied. The pathology of the spinal cord in HD is comparatively less well documented.OBJECTIVE: ... read more
- CalDAG-GEFI mediates striatal cholinergic modulation of dendritic excitability, synaptic plasticity and psychomotor behaviors [NCBI]Neurobiol Dis. 2021 Aug 6:105473. doi: 10.1016/j.nbd.2021.105473. Online ahead of print.ABSTRACTCalDAG-GEFI (CDGI) is a protein highly enriched in the striatum, particularly in principle spiny projection neurons (SPNs). CDGI is strongly down-regulated in two hyperkinetic conditions related to striatal dysfunction: Huntington's disease and levodopa-induced dyskinesia in Parkinson's disease. We demonstrate that ... read more
- Phytochemicals targeting oxidative stress, interconnected neuroinflammatory and neuroapoptotic pathways following radiation [NCBI]Curr Neuropharmacol. 2021 Aug 8. doi: 10.2174/1570159X19666210809103346. Online ahead of print.ABSTRACTThe radiation for therapeutic purposes has shown positive effects in different contexts; however, it can increase the risk of many age-related and neurodegenerative diseases such as Alzheimer's disease (AD), amyotrophic lateral sclerosis (ALS), Huntington's disease (HD), and Parkinson's disease (PD). ... read more
- Rapid quantification of extracellular neurotransmitters in mouse brain by PESI/MS/MS and longitudinal data analysis using the R and Stan-based Bayesian state-space model [NCBI]Talanta. 2021 Nov 1;234:122620. doi: 10.1016/j.talanta.2021.122620. Epub 2021 Jun 17.ABSTRACTWe developed a methodology for rapid quantification of extracellular neurotransmitters in mouse brain by PESI/MS/MS and longitudinal data analysis using the R and Stan-based Bayesian state-space model. We performed a rapid analysis for quantifying extracellular l-glutamic acid (L-Glu) and gamma-aminobutyric acid ... read more
- Early onset Alzheimer's disease – a case study [NCBI]Psychiatr Pol. 2021 Apr 30;55(2):323-330. doi: 10.12740/PP/OnlineFirst/114122. Epub 2021 Apr 30.ABSTRACTDementia syndromes constitute problem not only for the elderly. Early-onset dementia (EOD) starts below the age of 65 years. It accounts for 4-10% of all cases of dementia. EOD has significant psychosocial consequences because it affects people in their most ... read more
- The Therapeutic Potential of Celastrol in Central Nervous System Disorders: Highlights from In Vitro and In Vivo Approaches [NCBI]Molecules. 2021 Aug 3;26(15):4700. doi: 10.3390/molecules26154700.ABSTRACTCelastrol, the most abundant compound derived from the root of Tripterygium wilfordii, largely used in traditional Chinese medicine, has shown preclinical and clinical efficacy for a broad range of disorders, acting via numerous mechanisms, including the induction of the expression of several neuroprotective factors, the ... read more
- Emerging hiPSC Models for Drug Discovery in Neurodegenerative Diseases [NCBI]Int J Mol Sci. 2021 Jul 30;22(15):8196. doi: 10.3390/ijms22158196.ABSTRACTNeurodegenerative diseases affect millions of people worldwide and are characterized by the chronic and progressive deterioration of neural function. Neurodegenerative diseases, such as Alzheimer's disease (AD), Parkinson's disease (PD), amyotrophic lateral sclerosis (ALS), and Huntington's disease (HD), represent a huge social and ... read more
- Copper, Iron, and Manganese Toxicity in Neuropsychiatric Conditions [NCBI]Int J Mol Sci. 2021 Jul 22;22(15):7820. doi: 10.3390/ijms22157820.ABSTRACTCopper, manganese, and iron are vital elements required for the appropriate development and the general preservation of good health. Additionally, these essential metals play key roles in ensuring proper brain development and function. They also play vital roles in the central nervous ... read more
- Pallidal degenerations and related disorders: an update [NCBI]J Neural Transm (Vienna). 2021 Aug 7. doi: 10.1007/s00702-021-02392-2. Online ahead of print.ABSTRACTNeurodegenerative disorders involving preferentially the globus pallidus, its efferet and afferent circuits and/or related neuronal systems are rare. They include a variety of both familial and sporadic progressive movement disorders, clinically manifesting as choreoathetosis, dystonia, Parkinsonism, akinesia or ... read more
- INTEGRATIVE NETWORK LEARNING FOR MULTI-MODALITY BIOMARKER DATA [NCBI]Ann Appl Stat. 2021 Mar;15(1):64-87. doi: 10.1214/20-aoas1382. Epub 2021 Mar 18.ABSTRACTThe biomarker networks measured by different modalities of data (e.g., structural magnetic resonance imaging (sMRI), diffusion tensor imaging (DTI)) may share the same true underlying biological model. In this work, we propose a node-wise biomarker graphical model to leverage the ... read more
- AMPK in the brain: its roles in glucose and neural metabolism [NCBI]FEBS J. 2021 Aug 6. doi: 10.1111/febs.16151. Online ahead of print.ABSTRACTThe adenosine monophosphate-activated protein kinase (AMPK) is an integrative metabolic sensor that maintains energy balance at the cellular level and plays an important role in orchestrating inter-tissue metabolic signaling. AMPK regulates cell survival, metabolism and cellular homeostasis basally as well ... read more
- Huntingtin and Its Role in Mechanisms of RNA-Mediated Toxicity [NCBI]Toxins (Basel). 2021 Jul 14;13(7):487. doi: 10.3390/toxins13070487.ABSTRACTHuntington's disease (HD) is caused by a CAG-repeat expansion mutation in the Huntingtin (HTT) gene. It is characterized by progressive psychiatric and neurological symptoms in combination with a progressive movement disorder. Despite the ubiquitous expression of HTT, pathological changes occur quite selectively in the ... read more
- Modeling Manifest Huntington's Disease Prevalence Using Diagnosed Incidence and Survival Time [NCBI]Neuroepidemiology. 2021 Jul 15:1-8. doi: 10.1159/000516767. Online ahead of print.ABSTRACTINTRODUCTION: Understanding the epidemiology of Huntington's disease (HD) is key to assessing disease burden and the healthcare resources required to meet patients' needs. We aimed to develop and validate a model to estimate the diagnosed prevalence of manifest HD by the ... read more
- [11C]CHDI-626, a PET Tracer Candidate for Imaging Mutant Huntingtin Aggregates with Reduced Binding to AD Pathological Proteins [NCBI]J Med Chem. 2021 Aug 5. doi: 10.1021/acs.jmedchem.1c00667. Online ahead of print.ABSTRACTThe expanded polyglutamine-containing mutant huntingtin (mHTT) protein is implicated in neuronal degeneration of medium spiny neurons in Huntington's disease (HD) for which multiple therapeutic approaches are currently being evaluated to eliminate or reduce mHTT. Development of effective and orthogonal ... read more
- Huntington's Disease With Psychotic Features [NCBI]Prim Care Companion CNS Disord. 2021 Aug 5;23(4):20l02745. doi: 10.4088/PCC.20l02745.NO ABSTRACTPMID:34352939 | DOI:10.4088/PCC.20l02745 ... read more
- Systems modeling of metabolic dysregulation in neurodegenerative diseases [NCBI]Curr Opin Pharmacol. 2021 Aug 2;60:59-65. doi: 10.1016/j.coph.2021.06.012. Online ahead of print.ABSTRACTNeurodegenerative diseases (NDDs) encompass a wide range of conditions that arise owing to progressive degeneration and the ultimate loss of nerve cells in the brain and peripheral nervous system. NDDs such as Alzheimer's, Parkinson's, and Huntington's diseases negatively impact ... read more
- Endophenotypical drift in Huntington's disease: a 5-year follow-up study [NCBI]Orphanet J Rare Dis. 2021 Aug 3;16(1):340. doi: 10.1186/s13023-021-01967-2.ABSTRACTBACKGROUND: Huntington's disease (HD) is clinically characterized by progressing motor, cognitive and psychiatric symptoms presenting as varying phenotypes within these three major symptom domains. The disease is caused by an expanded CAG repeat tract in the huntingtin gene and the pathomechanism leading ... read more
- Impaired Restoration of Global Protein Synthesis Contributes to Increased Vulnerability to Acute ER Stress Recovery in Huntington's Disease [NCBI]Cell Mol Neurobiol. 2021 Aug 4. doi: 10.1007/s10571-021-01137-9. Online ahead of print.ABSTRACTNeurons are susceptible to different cellular stresses and this vulnerability has been implicated in the pathogenesis of Huntington's disease (HD). Accumulating evidence suggest that acute or chronic stress, depending on its duration and severity, can cause irreversible cellular damages ... read more
- Extending the spectrum of non-motor symptoms with olfaction in pre-motor Huntington's disease – a pilot study [NCBI]Neurodegener Dis. 2021 Jul 9:1. doi: 10.1159/000518136. Online ahead of print.NO ABSTRACTPMID:34348324 | DOI:10.1159/000518136 ... read more
- Genome-wide DNA methylation and gene expression analyses in monozygotic twins identify potential biomarkers of depression [NCBI]Transl Psychiatry. 2021 Aug 2;11(1):416. doi: 10.1038/s41398-021-01536-y.ABSTRACTDepression is currently the leading cause of disability around the world. We conducted an epigenome-wide association study (EWAS) in a sample of 58 depression score-discordant monozygotic twin pairs, aiming to detect specific epigenetic variants potentially related to depression and further integrate with gene expression ... read more
- Correction for Morozko et al., PIAS1 modulates striatal transcription, DNA damage repair, and SUMOylation with relevance to Huntington's disease [NCBI]Proc Natl Acad Sci U S A. 2021 Aug 10;118(32):e2112001118. doi: 10.1073/pnas.2112001118.NO ABSTRACTPMID:34341126 | DOI:10.1073/pnas.2112001118 ... read more
- Medicolegal Aspects of Huntington Disease [NCBI]J Am Acad Psychiatry Law. 2021 Aug 2:JAAPL.210008-21. doi: 10.29158/JAAPL.210008-21. Online ahead of print.ABSTRACTUnlawful behaviors have been reported in association with Huntington's disease (HD), although their overall prevalence and clinical significance remain unknown. Recognition of problematic behavior is limited by stigma and lack of routine clinical assessment, as well as ... read more
- How to Arrange Follow-Up Time-Intervals for Longitudinal Brain MRI Studies in Neurodegenerative Diseases [NCBI]Front Neurosci. 2021 Jul 15;15:682812. doi: 10.3389/fnins.2021.682812. eCollection 2021.ABSTRACTBACKGROUND: Longitudinal brain MRI monitoring in neurodegeneration potentially provides substantial insights into the temporal dynamics of the underlying biological process, but is time- and cost-intensive and may be a burden to patients with disabling neurological diseases. Thus, the conceptualization of follow-up time-intervals ... read more
- Utility of Huntington's Disease Assessments by Disease Stage: Floor/Ceiling Effects [NCBI]Front Neurol. 2021 Jul 15;12:595679. doi: 10.3389/fneur.2021.595679. eCollection 2021.ABSTRACTIntroduction: An understanding of the clinimetric properties of clinical assessments, including their constraints, is critical to sound clinical study and trial design. Utilizing data from Enroll-HD-a global, prospective HD observational study and clinical research platform-we examined several well-established HD clinical assessments across ... read more
- Hereditary Chorea Associated With and Aggravated by Systemic Lupus Erythematosus [NCBI]Cureus. 2021 Jun 28;13(6):e15992. doi: 10.7759/cureus.15992. eCollection 2021 Jun.ABSTRACTChorea is caused by a number of conditions, including genetic, metabolic derangements, infections, drugs, toxins, tumors, and disorders of the immune and inflammatory system of the body. Huntington's disease (HD) is the most common genetic cause of chorea. Systemic lupus erythematosus (SLE) ... read more
- Betulinic Acid Hydroxamate is Neuroprotective and Induces Protein Phosphatase 2A-Dependent HIF-1α Stabilization and Post-transcriptional Dephosphorylation of Prolyl Hydrolase 2 [NCBI]Neurotherapeutics. 2021 Aug 2. doi: 10.1007/s13311-021-01089-4. Online ahead of print.ABSTRACTHuntington's disease (HD) is a neurodegenerative disorder characterized by unwanted choreatic movements, behavioral and psychiatric disturbances, and dementia. The activation of the hypoxic response pathway through the pharmacological inhibition of hypoxia-inducing factor (HIF) prolyl-hydroxylases (PHDs) is a promising approach for neurodegenerative ... read more
- The neuroprotective potential of carotenoids in vitro and in vivo [NCBI]Phytomedicine. 2021 Jul 21;91:153676. doi: 10.1016/j.phymed.2021.153676. Online ahead of print.ABSTRACTBACKGROUND: Despite advances in research on neurodegenerative diseases, the pathogenesis and treatment response of neurodegenerative diseases remain unclear. Recent studies revealed a significant role of carotenoids to treat neurodegenerative diseases. The aim of this study was to systematically review the neuroprotective ... read more
- Oxygen-glucose deprivation in neurons: implications for cell transplantation therapies [NCBI]Prog Neurobiol. 2021 Jul 30:102126. doi: 10.1016/j.pneurobio.2021.102126. Online ahead of print.ABSTRACTCell replacement therapies hold the potential to restore neuronal networks compromised by neurodegenerative diseases (such as Parkinson's disease or Huntington's disease), or focal tissue damage (via a stroke or spinal cord injury). Despite some promising results achieved to date, transplanted ... read more
- FAN1-MLH1 interaction affects repair of DNA interstrand cross-links and slipped-CAG/CTG repeats [NCBI]Sci Adv. 2021 Jul 30;7(31):eabf7906. doi: 10.1126/sciadv.abf7906. Print 2021 Jul.ABSTRACTFAN1, a DNA structure-specific nuclease, interacts with MLH1, but the repair pathways in which this complex acts are unknown. FAN1 processes DNA interstrand crosslinks (ICLs) and FAN1 variants are modifiers of the neurodegenerative Huntington's disease (HD), presumably by regulating HD-causing CAG ... read more
- Factor Structure of the Repeatable Battery for the Assessment of Neuropsychological Status in Huntington's Disease [NCBI]Arch Clin Neuropsychol. 2021 Jun 28:acab050. doi: 10.1093/arclin/acab050. Online ahead of print.ABSTRACTOBJECTIVE: The Repeatable Battery for the Assessment of Neuropsychological Status is a commonly used neuropsychological screening tool that is useful in a Huntington's disease (HD) population given its relatively brief administration time and assessment of multiple cognitive domains. Although ... read more
- mGluR2/3 Activation Improves Motor Performance and Reduces Pathology in heterozygous zQ175 Huntington's Disease Mice [NCBI]J Pharmacol Exp Ther. 2021 Jul 30:JPET-AR-2021-000735. doi: 10.1124/jpet.121.000735. Online ahead of print.ABSTRACTHuntington's Disease (HD) is an autosomal dominant neurodegenerative disease that leads to progressive motor impairment with no available disease-modifying treatments. Current evidence indicates that exacerbated postsynaptic glutamate signaling in the striatum plays a key role in the pathophysiology ... read more
- Mutant Huntingtin Impairs Pancreatic beta-cells by Recruiting IRS-2 and Disturbing the PI3K/AKT/FoxO1 Signaling Pathway in Huntington's Disease [NCBI]J Mol Neurosci. 2021 Jul 31. doi: 10.1007/s12031-021-01869-9. Online ahead of print.ABSTRACTPatients with Huntington's disease (HD) have an increased incidence of diabetes. However, the molecular mechanisms of pancreatic β-cell dysfunction have not been entirely clarified. Revealing the pathogenesis of diabetes can provide a novel understanding of the onset and progression ... read more
- An updated review on the versatile role of chrysin in neurological diseases: Chemistry, pharmacology, and drug delivery approaches [NCBI]Biomed Pharmacother. 2021 Jul 16;141:111906. doi: 10.1016/j.biopha.2021.111906. Online ahead of print.ABSTRACTNeurological diseases are responsible for a large number of morbidities and mortalities in the world. Flavonoids are phytochemicals that possess various health-promoting impacts. Chrysin, a natural flavonoid isolated from diverse fruits, vegetables, and even mushrooms, has several pharmacological activities comprising ... read more
- Mitophagy in Huntington's disease [NCBI]Neurochem Int. 2021 Jul 27:105147. doi: 10.1016/j.neuint.2021.105147. Online ahead of print.ABSTRACTHuntington's disease (HD), as well as Parkinson's disease and Alzheimer's disease, belong to a group of neurodegenerative diseases characterized by common features, such as the progressive loss of neurons and the presence of pathogenic forms of misfolded protein aggregates. A ... read more
- Safety and feasibility of research lumbar puncture in Huntington's disease: the HDClarity cohort and bioresource [MedRxiv_Neurology]Background: Biomarkers are needed to monitor disease progression, target engagement and efficacy in Huntington's disease (HD). Cerebrospinal fluid (CSF) is an ideal medium to research such biomarkers due to its proximity to the brain. Objectives: To investigate the safety and feasibility of research lumbar punctures (LP) in HD. Methods: HDClarity ... read more
- A phenolic-rich extract from Ugni molinae berries reduces abnormal protein aggregation in a cellular model of Huntington's disease [NCBI]PLoS One. 2021 Jul 29;16(7):e0254834. doi: 10.1371/journal.pone.0254834. eCollection 2021.ABSTRACTAccumulation of misfolded proteins in the brain is a common hallmark of most age-related neurodegenerative diseases. Previous studies from our group identified the presence of anti-inflammatory and antioxidant compounds in leaves derived from the Chilean berry Ugni molinae (murtilla), in addition to ... read more
- Development of a High-Performance Multiparametric MRI Oropharyngeal Primary Tumor Auto-Segmentation Deep Learning Model and Investigation of Input Channel Effects: Results from a Prospective Imaging Registry [MedRxiv_Radiology_and_Imaging]Background and Purpose: Oropharyngeal cancer (OPC) primary gross tumor volume (GTVp) segmentation is crucial for radiotherapy. Multiparametric MRI (mpMRI) is increasingly used for OPC adaptive radiotherapy but relies on manual segmentation. Therefore, we constructed mpMRI deep learning (DL) OPC GTVp auto-segmentation models and determined the impact of input channels on ... read more
- Neurodegenerative movement disorders: an epigenetics perspective and promise for the future [NCBI]Neuropathol Appl Neurobiol. 2021 Jul 27. doi: 10.1111/nan.12757. Online ahead of print.ABSTRACTNeurodegenerative movement disorders (NMDs) are age dependent disorders that are characterised by the degeneration and loss of neurons, typically accompanied by pathological accumulation of different protein aggregates in the brain, which lead to motor symptoms. NMDs include Parkinson's disease, ... read more
- Emerging therapeutics in Huntington's Disease [NCBI]Expert Opin Emerg Drugs. 2021 Jul 28. doi: 10.1080/14728214.2021.1962285. Online ahead of print.ABSTRACTINTRODUCTION: Huntington's Disease is a neurodegenerative disease that is characterized by motor dysfunction, behavioral/psychiatric symptoms, and cognitive impairment. Because of the lack of availability of curative or disease modifying treatments, much of clinical practice in HD care to ... read more
- Mutant huntingtin crosses the neuromuscular junction and causes transmission-selective pathological alterations in huntingtin expressing myotubes [BioRxiv_Neuroscience]Skeletal muscle dysfunction, wasting and synaptic pathology is a hallmark of Huntingtons disease (HD). Similar as for the nervous system the pathological lesions and clinical symptoms progressively worsen with disease coarse. Cell-to-cell transmission of toxic mutant huntingtin (mHTT) has been shown to occur and could be a potential explanation for ... read more
- Assessment of EGFP-Q74 degradation for the measurement of autophagic flux [NCBI]Methods Cell Biol. 2021;165:31-38. doi: 10.1016/bs.mcb.2020.10.018. Epub 2021 Apr 27.ABSTRACTAutophagy plays a major role in physiological and pathological processes. The quantitation of the abundance of autophagy-specific substrates constitutes an efficient strategy for assessing autophagic activity. Here, we provide a detailed protocol for quantifying the decay of a fusion protein composed ... read more
- Publisher Correction: Questions swirl around failures of disease-modifying Huntington's drugs [NCBI]Nat Biotechnol. 2021 Jul 26. doi: 10.1038/s41587-021-01017-z. Online ahead of print.NO ABSTRACTPMID:34312502 | DOI:10.1038/s41587-021-01017-z ... read more
- Neuroprotective effects of rutin on ASH neurons in Caenorhabditis elegans model of Huntington's disease [NCBI]Nutr Neurosci. 2021 Jul 26:1-14. doi: 10.1080/1028415X.2021.1956254. Online ahead of print.ABSTRACTHuntington's disease (HD) is an autosomal dominant, progressive neurodegenerative disease. It occurs due to a mutated huntingtin gene that contains an abnormal expansion of cytosine-adenine-guanine repeats, leading to a variable-length N-terminal polyglutamine (polyQ) chain. The mutation confers toxic functions to ... read more
- Gene expression profiles of myopic mouse scleral fibroblasts: a bioinformatics analysis based on single-cell RNA sequencing [NCBI]Nan Fang Yi Ke Da Xue Xue Bao. 2021 Jul 20;41(7):1087-1092. doi: 10.12122/j.issn.1673-4254.2021.07.18.ABSTRACTOBJECTIVE: To investigate the changes in gene expression profiles of mouse scleral fibroblasts after myopia using single-cell RNA sequencing technology and explore the mechanism of dysfunction of the scleral fibroblasts in myopia.METHODS: Normal healthy C57BL/6J mice were randomly ... read more
- Mild Cognitive Impairment as an Early Landmark in Huntington's Disease [NCBI]Front Neurol. 2021 Jul 7;12:678652. doi: 10.3389/fneur.2021.678652. eCollection 2021.ABSTRACTAs one of the clinical triad in Huntington's disease (HD), cognitive impairment has not been widely accepted as a disease stage indicator in HD literature. This work aims to study cognitive impairment thoroughly for prodromal HD individuals with the data from a ... read more
- Chronic methamphetamine uncovers a circadian rhythm in multiple-unit neural activity in the dorsal striatum which is independent of the suprachiasmatic nucleus [NCBI]Neurobiol Sleep Circadian Rhythms. 2021 Jun 25;11:100070. doi: 10.1016/j.nbscr.2021.100070. eCollection 2021 Nov.ABSTRACTThe dorsal striatum forms part of the basal ganglia circuit that is a major regulator of voluntary motor behavior. Dysfunction in this circuit is a critical factor in the pathology of neurological (Parkinson's and Huntington's disease) as well as ... read more
- The clinical, imaging and biological features of psychosis in Han Chinese patients with Huntington's disease [NCBI]J Psychiatr Res. 2021 Jul 14;141:333-338. doi: 10.1016/j.jpsychires.2021.07.024. Online ahead of print.ABSTRACTHuntington's disease (HD) is an autosomal dominant neurodegenerative disease involving motor, cognitive and psychiatric disturbances. HD patients with psychosis symptoms usually have bad prognosis. It is of great significance to explore the clinical, imaging and biological characteristics of HD ... read more
- Accelerated expansion of pathogenic mitochondrial DNA heteroplasmies in Huntington's disease [NCBI]Proc Natl Acad Sci U S A. 2021 Jul 27;118(30):e2014610118. doi: 10.1073/pnas.2014610118.ABSTRACTMitochondrial dysfunction is found in the brain and peripheral tissues of patients diagnosed with Huntington's disease (HD), an irreversible neurodegenerative disease of which aging is a major risk factor. Mitochondrial function is encoded by not only nuclear DNA but ... read more
- An unusual presentation of Huntington's disease [NCBI]Clin Case Rep. 2021 Jul 16;9(7):e04547. doi: 10.1002/ccr3.4547. eCollection 2021 Jul.ABSTRACTWe describe the case of a 59-year-old woman who exhibited psychotic symptoms, cognitive dysfunction, and restlessness. While the clinical picture and 18F-FDG PET/CT suggested the presence of a tauopathy, especially frontotemporal dementia or progressive supranuclear palsy, genetic testing eventually revealed ... read more
- Selective Neuron Vulnerability in Common and Rare Diseases-Mitochondria in the Focus [NCBI]Front Mol Biosci. 2021 Jun 30;8:676187. doi: 10.3389/fmolb.2021.676187. eCollection 2021.ABSTRACTMitochondrial dysfunction is a central feature of neurodegeneration within the central and peripheral nervous system, highlighting a strong dependence on proper mitochondrial function of neurons with especially high energy consumptions. The fitness of mitochondria critically depends on preservation of distinct processes, ... read more
- Clinical Spectrum of Movement Disorders in Neurology Inpatients in a Tertiary Care Centre [NCBI]J Neurosci Rural Pract. 2021 Jul;12(3):581-585. doi: 10.1055/s-0041-1730086. Epub 2021 May 10.ABSTRACTBackground Little data are available on the spectrum of movement disorders in inpatients, particularly those admitted in neurology specialty. This may be related to the fact that patients presenting with movement disorders are usually evaluated from outpatient clinics. Objective ... read more
- Immortalized striatal precursor neurons from Huntington's disease patient-derived iPS cells as a platform for target identification and screening for experimental therapeutics [NCBI]Hum Mol Genet. 2021 Jul 22:ddab200. doi: 10.1093/hmg/ddab200. Online ahead of print.ABSTRACTWe have previously established Induced Pluripotent Stem cell (iPSC) models of Huntington's Disease (HD), demonstrating CAG-repeat-expansion-dependent cell biological changes and toxicity. However, the current differentiation protocols are cumbersome and time consuming, making preparation of large quantities of cells for ... read more
- Disparities in Palliative Care Utilization Among Hospitalized People With Huntington Disease: A National Cross-Sectional Study [NCBI]Am J Hosp Palliat Care. 2021 Jul 22:10499091211034419. doi: 10.1177/10499091211034419. Online ahead of print.ABSTRACTBACKGROUND: People with Huntington's disease (HD) often become institutionalized and more frequently die away from the home setting. The reasons behind differences in end-of-life care are poorly understood. Less than 5% of people with HD report utilization ... read more
- Greater time in bed and less physical activity associate with poorer cognitive functioning performance in Huntington's disease [NCBI]Neuropsychology. 2021 Jul 22. doi: 10.1037/neu0000757. Online ahead of print.ABSTRACTObjective: This study aimed to investigate how sleep and physical activity habits related to cognitive functioning, in naturalistic settings, in early Huntington's disease (HD). Method: Forty-two participants with the expanded HD repeat (20 manifest, 22 premanifest) and 29 healthy controls wore ... read more
- Meaning and purpose in Huntington's disease: a longitudinal study of its impact on quality of life [NCBI]Ann Clin Transl Neurol. 2021 Jul 20. doi: 10.1002/acn3.51424. Online ahead of print.ABSTRACTOBJECTIVE: Previous work in Huntington's disease (HD) has shown that a sense of meaning and purpose (M&P) is positively associated with positive affect and well-being (PAW); however, it was unknown whether HD-validated patient-reported outcomes (PROs) influence this association ... read more
- Abnormal Recovery from Acute Stress in Huntingtons Disease Mice [BioRxiv_Neuroscience]Introduction: Comparisons of hypothalamic dysfunction between Huntingtons Disease (HD) patients and rodent models of HD have not always yielded similar results. Cortisol levels in HD patients have been contradictory, with reports ranging from hypo- to hypercorticoidism of morning measurements. Initial reports of major elevations in circulating corticosterone levels in the ... read more
- "Huntington disease-like phenotype in a patient with ANO3 mutation" Expert commentary [NCBI]Parkinsonism Relat Disord. 2021 Jul 14:S1353-8020(21)00258-3. doi: 10.1016/j.parkreldis.2021.07.009. Online ahead of print.NO ABSTRACTPMID:34281767 | DOI:10.1016/j.parkreldis.2021.07.009 ... read more
- SQSTM1/p62 droplet -mediated autophagosome formation:insights into Huntington disease [NCBI]Autophagy. 2021 Jul 19:1-4. doi: 10.1080/15548627.2021.1953820. Online ahead of print.ABSTRACTHuntington disease (HD) manifests a unique macroautophagy/autophagy defect: the presense of cytosolic autophagosomes without substrates or so-called "empty" autophagosomes. It was proposed that mutant HTT (huntingtin; mHTT) disrupts cargo recognition by the selective autophagy receptor SQSTM1/p62 thus leading to the failure ... read more
- Huntington's disease in psychiatric practice [NCBI]Zh Nevrol Psikhiatr Im S S Korsakova. 2021;121(6):81-86. doi: 10.17116/202112106181.ABSTRACTHuntington's disease, or Huntington's chorea, is considered as an orphan disease. However, chorea is not the single, and in many cases, not the most severe sign of this disease. Along with motor symptoms, psychiatric symptoms make a significant contribution to patient ... read more
- Toxocara canis Infection Alters lncRNA and mRNA Expression Profiles of Dog Bone Marrow [NCBI]Front Cell Dev Biol. 2021 Jun 30;9:688128. doi: 10.3389/fcell.2021.688128. eCollection 2021.ABSTRACTBone marrow is the main hematopoietic organ that produces red blood cells, granulocytes, monocyte/macrophages, megakaryocytes, lymphocytes, and myeloid dendritic cells. Many of these cells play roles in the pathogenesis of Toxocara canis infection, and understanding how infection alters the dynamics ... read more
- Purinergic Signaling in the Pathophysiology and Treatment of Huntington's Disease [NCBI]Front Neurosci. 2021 Jul 1;15:657338. doi: 10.3389/fnins.2021.657338. eCollection 2021.ABSTRACTHuntington's disease (HD) is a devastating, progressive, and fatal neurodegenerative disorder inherited in an autosomal dominant manner. This condition is characterized by motor dysfunction (chorea in the early stage, followed by bradykinesia, dystonia, and motor incoordination in the late stage), psychiatric disturbance, ... read more
- Juvenile Huntington's Disease and Other PolyQ Diseases, Update on Neurodevelopmental Character and Comparative Bioinformatic Review of Transcriptomic and Proteomic Data [NCBI]Front Cell Dev Biol. 2021 Jul 1;9:642773. doi: 10.3389/fcell.2021.642773. eCollection 2021.ABSTRACTPolyglutamine (PolyQ) diseases are neurodegenerative disorders caused by the CAG repeat expansion mutation in affected genes resulting in toxic proteins containing a long chain of glutamines. There are nine PolyQ diseases: Huntington's disease (HD), spinocerebellar ataxias (types 1, 2, 3, ... read more
- Is TGF-beta1 a Biomarker of Huntington's Disease Progression? [NCBI]J Clin Med. 2021 Jul 5;10(13):3001. doi: 10.3390/jcm10133001.ABSTRACTHuntington's disease (HD) is an autosomal dominant genetic disease that can be divided into preclinical and symptomatic stages. Due to the diverse HD phenotype, there is an urgent need to identify markers that would independently assess its severity. The aim of this study ... read more
- Neurobiological and Psychosocial Correlates of Communication Between Huntington's Disease Patients and Their Offspring [NCBI]J Neuropsychiatry Clin Neurosci. 2021 Jul 19:appineuropsych20120309. doi: 10.1176/appi.neuropsych.20120309. Online ahead of print.ABSTRACTOBJECTIVE: Huntington's disease (HD) is an autosomal dominant neurodegenerative disease that presents significant challenges to family communication. The investigators examined observations of communication between parents with HD and their offspring talking about the challenges of HD and explored ... read more
