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CNS Neurosci Ther. 2021 Dec 3. doi: 10.1111/cns.13768. Online ahead of print.ABSTRACTAIMS: The family of kynurenine pathway (KP) metabolites includes compounds produced along two arms of the path and acting in clearly opposite ways. The equilibrium between neurotoxic kynurenines, such as 3-hydroxykynurenine (3-HK) or quinolinic acid (QUIN), and neuroprotective kynurenic ... read more

Huntington's disease (HD) is a devastating monogenic neurodegenerative disease characterized by early, selective pathology in the basal ganglia despite the ubiquitous expression of mutant huntingtin. The molecular mechanisms underlying this region-specific neuronal degeneration and how these relate to the development of early cognitive phenotypes are poorly understood. Here, we show ... read more

Sleep Med. 2021 Nov 12:S1389-9457(21)00548-7. doi: 10.1016/j.sleep.2021.10.035. Online ahead of print.ABSTRACTBACKGROUND: Huntington's disease (HD) is a progressive neurodegenerative disorder characterised by chorea, cognitive impairment, psychiatric and behavioral disturbances. Sleep disturbances including reduced REM sleep have been observed in HD.OBJECTIVES: The aim of the study was to study the polysomnography findings ... read more

Curr Top Med Chem. 2021 Dec 1. doi: 10.2174/1568026621666211201150217. Online ahead of print.ABSTRACTNeurodegeneration is a syndrome that occurs through the loss of the neuronal system's structure and function. In the 21st century, major health issues are related to cognitive impairment and neurological disorders such as autism, learning disabilities, Huntington's, cerebral ... read more

Curr Neuropharmacol. 2021 Nov 30. doi: 10.2174/1570159X19666211201094608. Online ahead of print.ABSTRACTHuntington's disease (HD) is a neurodegenerative genetic disorder caused by a CAG repeat expansion in the huntingtin gene. HD causes motor, cognitive, and behavioral dysfunction. Since no existing treatment affects the course of this disease, new treatments are needed. Inflammation ... read more

Methods Mol Biol. 2022;2402:227-241. doi: 10.1007/978-1-0716-1843-1_18.ABSTRACTProtein misfolding diseases, like Alzheimer's, Parkinson's, and Huntington's disease, are associated with misfolded protein aggregation. Alzheimer's disease is related to a progressive neuronal death induced by small amyloid β oligomers. Here, we describe the procedure to prepare and identify different types of small toxic amyloid ... read more

Biomed Chromatogr. 2021 Dec 1:e5291. doi: 10.1002/bmc.5291. Online ahead of print.ABSTRACTCytochrome P450 46A1 (CYP46A1) is a key enzyme responsible for metabolizing cholesterol to 24-hydroxycholesterol in brain, which might be served as a therapeutic target for several neurodegenerative disorders including Parkinson's disease (PD), Alzheimer's disease (AD), and Huntington's disease (HD). However, ... read more

J Palliat Med. 2021 Nov 30. doi: 10.1089/jpm.2021.0308. Online ahead of print.ABSTRACTContext: Huntington's disease (HD) is a neurodegenerative disorder characterized by mid-life onset, cognitive decline, and behavioral disturbance. Objective: We conducted a review of the end-of-life (EOL) experience of HD patients and their families. Methods: We searched 5 electronic databases. ... read more

Neurobiol Dis. 2021 Nov 27:105574. doi: 10.1016/j.nbd.2021.105574. Online ahead of print.ABSTRACTHuntington's disease (HD) is a heritable, fatal neurodegenerative disorder caused by a mutation in the Huntingtin gene. It is characterized by chorea, as well as cognitive and psychiatric symptoms. Histopathologically, there is a massive loss of striatal projection neurons and ... read more

Asian J Pharm Sci. 2021 Sep;16(5):533-550. doi: 10.1016/j.ajps.2021.02.006. Epub 2021 Apr 2.ABSTRACTLong non coding RNAs (lncRNAs) are non-protein or low-protein coding transcripts that contain more than 200 nucleotides. They representing a large share of the cell's transcriptional output, demonstrate functional attributes viz. tissue-specific expression, determination of cell fate, controlled expression, ... read more

Nucleic Acids Res. 2021 Nov 25:gkab1126. doi: 10.1093/nar/gkab1126. Online ahead of print.ABSTRACTOligonucleotides is an emerging class of chemically-distinct therapeutic modalities, where extensive chemical modifications are fundamental for their clinical applications. Inter-nucleotide backbones are critical to the behaviour of therapeutic oligonucleotides, but clinically explored backbone analogues are, effectively, limited to phosphorothioates. ... read more

Cogn Behav Neurol. 2021 Dec 2;34(4):295-302. doi: 10.1097/WNN.0000000000000286.ABSTRACTBACKGROUND: Apathy is a prevalent behavioral syndrome of Huntington disease (HD) that can result in severe loss of function for the individual with HD and substantial caregiver distress. Research-based evidence of apathy is characterized by methodological differences, and there is a deficiency in ... read more

Huntington's disease (HD) is a devastating neurodegenerative disease that primarily affects the striatum, a brain region that controls movement and some forms of cognition. Dysfunction and loss of medium spiny neurons of the striatum is accompanied by astrogliosis (increased astrocyte density and pathology). For decades, astrocytes were considered a homogeneous ... read more

Sheng Wu Gong Cheng Xue Bao. 2021 Nov 25;37(11):3757-3780. doi: 10.13345/j.cjb.210253.ABSTRACTThe human intestinal flora is a highly diverse ecosystem composed of trillions of bacteria. The imbalance of the flora is related to a variety of diseases. The intestinal flora interacts with the nervous system bidirectionally in many ways through the ... read more

Mov Disord. 2021 Nov 29. doi: 10.1002/mds.28855. Online ahead of print.ABSTRACTBACKGROUND: Huntington's disease (HD) is a rare neurodegenerative disorder with protean clinical manifestations. Its management is challenging, consisting mainly of off-label treatments.OBJECTIVES: The International Parkinson and Movement Disorder Society commissioned a task force to review and evaluate the evidence of ... read more

White matter (WM) alterations have been observed early in Huntington's disease (HD) progression but their role in the disease-pathophysiology remains unknown. We exploited ultra-strong-gradient MRI to tease apart contributions of myelin (with the magnetization transfer ratio), and axon density (with the restricted volume fraction from the Composite Hindered and Restricted ... read more

Int J Mol Sci. 2021 Nov 13;22(22):12288. doi: 10.3390/ijms222212288.ABSTRACTRecent research demonstrated pathological spreading of the disease-causing proteins from one focal point across other brain regions for some neurodegenerative diseases, such as Parkinson's and Alzheimer's disease. Spreading mediated by extracellular vesicles is one of the proposed disease-spreading mechanisms. Extracellular vesicles are ... read more

Brain Sci. 2021 Nov 2;11(11):1455. doi: 10.3390/brainsci11111455.ABSTRACTFacial expression is a key aspect in observational scales developed to improve pain assessment in individuals with cognitive impairments. Although these scales are used internationally in individuals with different types of cognitive impairments, it is not known whether observing facial expressions of pain might ... read more

Biomedicines. 2021 Oct 20;9(11):1499. doi: 10.3390/biomedicines9111499.ABSTRACTPolyglutamine (polyQ) ataxias are a heterogenous group of neurological disorders all caused by an expanded CAG trinucleotide repeat located in the coding region of each unique causative gene. To date, polyQ ataxias encompass six disorders: spinocerebellar ataxia types 1, 2, 3, 6, 7, and 17 ... read more

Pharmaceutics. 2021 Nov 8;13(11):1897. doi: 10.3390/pharmaceutics13111897.ABSTRACTNeurodegenerative diseases (NDs) represent a heterogeneous group of aging-related disorders featured by progressive impairment of motor and/or cognitive functions, often accompanied by psychiatric disorders. NDs are denoted as 'protein misfolding' diseases or proteinopathies, and are classified according to their known genetic mechanisms and/or the main ... read more

Genes (Basel). 2021 Nov 9;12(11):1776. doi: 10.3390/genes12111776.ABSTRACTNeurodegenerative diseases result in the progressive deterioration of the nervous system, with motor and cognitive impairments being the two most observable problems. Motor dysfunction could be caused by motor neuron diseases (MNDs) characterized by the loss of motor neurons, such as amyotrophic lateral sclerosis ... read more

Brain Sci. 2021 Oct 28;11(11):1437. doi: 10.3390/brainsci11111437.ABSTRACTMitochondria associated membranes (MAMs), as the name suggests, are the membranes that physically and biochemically connect mitochondria with endoplasmic reticulum. MAMs not only structurally but also functionally connect these two important organelles within the cell which were previously thought to exist independently. There are ... read more

Cells. 2021 Nov 15;10(11):3172. doi: 10.3390/cells10113172.ABSTRACTMitochondrial morphology and motility (mitochondrial dynamics) play a major role in the proper functioning of distant synapses. In Huntington's disease (HD), mitochondria become fragmented and less motile, but the mechanisms leading to these changes are not clear. Here, we found that collapsin response mediator protein ... read more

Int J Mol Sci. 2021 Nov 19;22(22):12499. doi: 10.3390/ijms222212499.ABSTRACTHuntington's disease (HD) is a rare neurodegenerative disorder caused by an expansion of CAG trinucleotide repeat located in the exon 1 of Huntingtin (HTT) gene in human chromosome 4. The HTT protein is ubiquitously expressed in the brain. Specifically, mutant HTT (mHTT) ... read more

Protein Pept Lett. 2021 Nov 24. doi: 10.2174/0929866528666211125114421. Online ahead of print.ABSTRACTThe failure of protein to correctly fold into its functional and unique three dimensional form leads to misfolded or partially folded protein. When these rogue proteins and polypeptides escape the quality control mechanism within the body, they result in ... read more

J Mol Neurosci. 2021 Nov 26. doi: 10.1007/s12031-021-01944-1. Online ahead of print.ABSTRACTIntercellular propagation of aggregated protein inclusions along actin-based tunneling nanotubes (TNTs) has been reported as a means of pathogenic spread in Alzheimer's, Parkinson's, and Huntington's diseases. Propagation of oligomeric-structured polyglutamine-expanded ataxin-1 (Atxn1[154Q]) has been reported in the cerebellum of ... read more

Curr Neuropharmacol. 2021 Jul 12. doi: 10.2174/1570159X19666210712152532. Online ahead of print.ABSTRACTNowadays, neurodegenerative diseases (NDs), such as Parkinson's disease (PD), Alzheimer's disease (AD), Huntington's disease (HD), and amyotrophic lateral sclerosis (ALS), represent a great challenge for different scientific fields, such as neuropharmacology, medicinal chemistry, molecular biology, and medicine, once all these ... read more

There is a lack of comprehensive understanding of breast cancer (BC) specific sEVs characteristics and composition on BC unique proteomic information from human samples. Here, we interrogated the proteomic landscape of sEVs in 167 serum samples from patients with BC, benign mammary disease (BD) and from healthy donors (HD). The ... read more

JMIR Res Protoc. 2021 Nov 24;10(11):e32327. doi: 10.2196/32327.ABSTRACTBACKGROUND: Evidence supports several countries introducing legislation to allow cannabis-based medicine as an adjunctive treatment for the symptomatic relief of chronic pain, chemotherapy-induced nausea, spasticity in multiple sclerosis (MS), epileptic seizures, depression, and anxiety. However, clinical trial participants do not represent the entire ... read more

High-density Electroencephalography (HD-EEG) has been proven to be the most accurate option to estimate the neural activity inside the brain. Although multiple studies report the effect of electrode number on source localization for specific sources and specific electrode configurations, the electrodes for each configuration have been manually selected to uniformly ... read more

Stem Cell Res Ther. 2021 Nov 22;12(1):585. doi: 10.1186/s13287-021-02653-7.ABSTRACTBACKGROUND: Human embryonic stem cells (hESCs) transplantation had shown to provide a potential source of cells in neurodegenerative disease studies and lead to behavioral recovery in lentivirus transfected or, toxin-induced Huntington's disease (HD) rodent model. Here, we aimed to observe if transplantation ... read more

Mol Neurobiol. 2021 Nov 23. doi: 10.1007/s12035-021-02638-w. Online ahead of print.ABSTRACTNeurological disorders are primarily diseases with sophisticated etiology that are always refractory and recrudescent. The major obstruction to effective therapies for neurological disorders is the poor understanding of their pathogenic mechanisms. CRISPR-Cas9 technology, which allows precise and effective gene editing ... read more

CNS Neurol Disord Drug Targets. 2021 Nov 19. doi: 10.2174/1871527320666211119122150. Online ahead of print.ABSTRACTBACKGROUND: Vitamins are the micronutrients required for boosting the immune system and managing any future infection. Vitamins are involved in neurogenesis, a defense mechanism working in neurons, metabolic reactions, neuronal survival, and neuronal transmission. Their deficiency leads ... read more

Front Aging Neurosci. 2021 Nov 4;13:722944. doi: 10.3389/fnagi.2021.722944. eCollection 2021.ABSTRACTBackground: For measuring the impact in clinical and scientific research, the citation count of the articles is used in the bibliometric analysis, although there is no comprehensive summary of neurodegenerative disease research. This study intends to provide the neuroscientists and investigators ... read more

Oxid Med Cell Longev. 2021 Nov 10;2021:5522981. doi: 10.1155/2021/5522981. eCollection 2021.ABSTRACTNeurodegenerative diseases (NDs) like Alzheimer's disease, multiple sclerosis, amyotrophic lateral sclerosis, Parkinson's disease, and Huntington's disease predominantly pose a significant socioeconomic burden. Characterized by progressive neural dysfunction coupled with motor or intellectual impairment, the pathogenesis of ND may result from ... read more

Front Aging Neurosci. 2021 Nov 3;13:766587. doi: 10.3389/fnagi.2021.766587. eCollection 2021.ABSTRACTNeurodegenerative diseases, namely Alzheimer's (AD), Parkinson's (PD), and Huntington's disease (HD) together with amyotrophic lateral sclerosis (ALS) and multiple sclerosis (MS), devastate millions of lives per year worldwide and impose an increasing socio-economic burden across nations. Consequently, these diseases occupy a ... read more

Front Aging Neurosci. 2021 Nov 5;13:736734. doi: 10.3389/fnagi.2021.736734. eCollection 2021.ABSTRACTHuntington's disease (HD) is an inherited neurodegenerative disorder characterized by neuronal loss and tissue atrophy mainly in the striatum and cortex. In the early stages of the disease, impairment of neuronal function, synaptic dysfunction and white matter loss precedes neuronal death ... read more

Hum Gene Ther. 2021 Nov 22. doi: 10.1089/hum.2021.221. Online ahead of print.ABSTRACTHuntington's Disease is a fatal neurodegenerative disorder caused by an inherited mutation in the huntingtin gene (HTT) comprising an expanded cytosine-adenine-guanine (CAG) trinucleotide repeat sequence that results in a pathogenic huntingtin protein. AAV gene therapy containing a primary artificial ... read more

Short tandem repeats (STRs) are tandemly repeated sequences of 1-6 bp motifs. STRs compose approximately 3% of the genome, and mutations at STR loci have been linked to dozens of human diseases including amyotrophic lateral sclerosis, Friedreich ataxia, Huntington disease, and fragile X syndrome. Improving our understanding of these mutations ... read more

Neurochem Res. 2021 Nov 20. doi: 10.1007/s11064-021-03488-7. Online ahead of print.ABSTRACTNeurodegenerative disorders are distinguished by the gradual deterioration of the nervous system's structure and function due to oxidative stress, mitochondrial dysfunction, protein misfolding, excitotoxicity, and neuroinflammation. Among these NDs, Alzheimer's disease, Huntington's disease, Parkinson's disease, and amyotrophic lateral sclerosis characterized ... read more

Acta Neuropathol. 2021 Nov 20. doi: 10.1007/s00401-021-02385-1. Online ahead of print.NO ABSTRACTPMID:34800149 | DOI:10.1007/s00401-021-02385-1 ... read more

Chembiochem. 2021 Nov 18. doi: 10.1002/cbic.202100533. Online ahead of print.ABSTRACTAbnormally expanded CAG/CTG repeat DNA sequences lead to a variety of neurological diseases, such as Huntington's disease. Here, we synthesized a cyclic pyrrole-imidazole polyamide (cPIP), which can bind to the minor groove of the CAG/CTG DNA sequence. The double-stranded DNA melting ... read more

Nat Commun. 2021 Nov 18;12(1):6666. doi: 10.1038/s41467-021-27056-3.ABSTRACTExtracellular vesicles (EVs) are biological nanoparticles with important roles in intercellular communication, and potential as drug delivery vehicles. Here we demonstrate a role for the glycolytic enzyme glyceraldehyde-3-phosphate dehydrogenase (GAPDH) in EV assembly and secretion. We observe high levels of GAPDH binding to the ... read more

Transl Neurodegener. 2021 Nov 15;10(1):46. doi: 10.1186/s40035-021-00272-z.ABSTRACTAmyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by selective, early degeneration of motor neurons in the brain and spinal cord. Motor neurons have long axonal projections, which rely on the integrity of neuronal cytoskeleton and mitochondria to regulate energy requirements for ... read more

J Neurosci. 2021 Nov 17;41(46):9483-9502. doi: 10.1523/JNEUROSCI.0620-21.2021.ABSTRACTUnderstanding how neurons of the striatum are formed and integrate into complex synaptic circuits is essential to provide insight into striatal function in health and disease. In this review, we summarize our current understanding of the development of striatal neurons and associated circuits with ... read more

Neuron. 2021 Nov 9:S0896-6273(21)00863-1. doi: 10.1016/j.neuron.2021.10.033. Online ahead of print.ABSTRACTAlthough the classic symptoms of Huntington's disease (HD) manifest in adulthood, neural progenitor cell behavior is already abnormal by 13 weeks' gestation. To determine how these developmental defects evolve, we turned to cell and mouse models. We found that layer II/III ... read more

Eur J Pharm Biopharm. 2021 Nov 15:S0939-6411(21)00276-9. doi: 10.1016/j.ejpb.2021.11.003. Online ahead of print.ABSTRACTHuntington's disease (HD) is a neurodegenerative disorder caused by a mutation in the huntingtin (HTT) gene, leading to a toxic version of the HTT protein. There are currently no disease-modifying therapies available. In this scenario, gene-based treatments for ... read more

Matrix Biol Plus. 2021 Oct 23;12:100089. doi: 10.1016/j.mbplus.2021.100089. eCollection 2021 Dec.ABSTRACTCellular adhesive connections directed by the extracellular matrix (ECM) and maintenance of cellular homeostasis by autophagy are seemingly disparate functions that are molecularly intertwined, each regulating the other. This is an emerging field in the brain where the interplay between ... read more

Neurodegener Dis Manag. 2021 Nov 17. doi: 10.2217/nmt-2021-0023. Online ahead of print.ABSTRACTHuntington's disease (HD) is an autosomal dominant neurodegenerative disorder with core clinical features of choreoathetosis, cognitive deficits and behavioral changes. It is a rare disorder, primarily affecting the Caucasian population, and rarely Asians. To date, there are only two ... read more

J Mov Disord. 2021 Nov 17. doi: 10.14802/jmd.21006. Online ahead of print.ABSTRACTHuntington's disease (HD) has become a target of the first clinical trials for gene therapy among movement disorders with a genetic origin. More than 100 clinical trials regarding HD have been tried, but all failed, although there were some ... read more

Disabil Rehabil. 2021 Nov 16:1-14. doi: 10.1080/09638288.2021.2002436. Online ahead of print.ABSTRACTPURPOSE: Research on breaking bad news (BBN) in healthcare has mostly focused on the doctor-patient interaction during a single consultation. However, it has been increasingly recognised that BBN is a wider process that also involves other healthcare professionals. This qualitative ... read more

Background: Cortico-cortical evoked potentials (CCEPs) recorded by stereo-electroencephalography (SEEG) are a valuable clinical tool to investigate brain reactivity and effective connectivity. However, these invasive recordings are spatially sparse since they depend on clinical needs. This sparsity hampers systematic comparisons across-subjects, the detection of the whole-brain spatiotemporal properties of CCEPs, as ... read more

iScience. 2021 Oct 14;24(11):103279. doi: 10.1016/j.isci.2021.103279. eCollection 2021 Nov 19.ABSTRACTPreclinical drug candidates are screened for their ability to ameliorate in vitro neuronal electrophysiology, and go/no-go decisions progress drugs to clinical trials based on population means across cells and animals. However, these measures do not mitigate clinical endpoint risk. Population-based modeling ... read more

Brain Commun. 2021 Oct 27;3(4):fcab261. doi: 10.1093/braincomms/fcab261. eCollection 2021.ABSTRACTNeurosecretory protein VGF (non-acronymic) belongs to the granin family of neuropeptides. VGF and VGF-derived peptides have been repeatedly identified in well-powered and well-designed multi-omic studies as dysregulated in neurodegenerative and psychiatric diseases. New therapeutics is urgently needed for these devastating and costly ... read more

Front Neurol. 2021 Oct 27;12:719442. doi: 10.3389/fneur.2021.719442. eCollection 2021.ABSTRACTBackground: Huntington's disease (HD) leads to altered gait patterns and reduced daily-living physical activity. Accurate measurement of daily-living walking that takes into account involuntary movements (e.g. chorea) is needed. Objective: To evaluate daily-living gait quantity and quality in HD, taking into account ... read more

J Clin Med. 2021 Nov 5;10(21):5181. doi: 10.3390/jcm10215181.ABSTRACTBrain-derived neurotrophic factor (BDNF) is involved in the survival and maturation of neurons, and also promotes and controls neurogenesis. Its levels are lowered in many neurodegenerative diseases, including Huntington's disease (HD). Clinical pictures of HD can be very diverse, which makes it difficult ... read more

Nat Commun. 2021 Nov 12;12(1):6579. doi: 10.1038/s41467-021-26684-z.ABSTRACTDespite the strong evidence linking the aggregation of the Huntingtin protein (Htt) to the pathogenesis of Huntington's disease (HD), the mechanisms underlying Htt aggregation and neurodegeneration remain poorly understood. Herein, we investigated the ultrastructural properties and protein composition of Htt cytoplasmic and nuclear inclusions ... read more

Oral Dis. 2021 Nov 12. doi: 10.1111/odi.14076. Online ahead of print.ABSTRACTOBJECTIVES: The objective of this systematic literature review was to provide a complete panorama of the oral manifestations of Huntington's disease (HD).MATERIALS AND METHODS: Databases were searched and original research studies or case report manuscripts up to May 2021 were ... read more

Physiol Genomics. 2021 Nov 12. doi: 10.1152/physiolgenomics.00066.2021. Online ahead of print.ABSTRACTBACKGROUND: Broad cellular functions and diseases including muscular dystrophy, arrhythmogenic right ventricular cardiomyopathy (ARVC5) and cancer are associated with transmembrane protein43 (TMEM43/LUMA).OBJECTIVE: The study aimed to investigate biological roles of TMEM43 through genetic regulation, gene pathways and gene networks, candidate ... read more

Front Psychol. 2021 Oct 25;12:722740. doi: 10.3389/fpsyg.2021.722740. eCollection 2021.ABSTRACTObjective: Chronic physical illness affects not only patients but also their partners. Dyadic coping (DC)-the ways couples cope in dealing with a stressor such as chronic illness-has received increased attention over the last three decades. The aim of the current study was ... read more

Ann Clin Transl Neurol. 2021 Nov 10. doi: 10.1002/acn3.51460. Online ahead of print.ABSTRACTFemale Huntington's disease (HD) patients have consistently shown a faster clinical worsening than male, but the underlying mechanisms responsible for this observation remain unknown. Here, we describe how sex modifies the impact of neurodegeneration on brain atrophy and ... read more

Nat Rev Neurol. 2021 Nov 10. doi: 10.1038/s41582-021-00577-7. Online ahead of print.ABSTRACTEndogenous biological clocks, orchestrated by the suprachiasmatic nucleus, time the circadian rhythms that synchronize physiological and behavioural functions in humans. The circadian system influences most physiological processes, including sleep, alertness and cognitive performance. Disruption of circadian homeostasis has deleterious ... read more

Nervenarzt. 2021 Nov 11. doi: 10.1007/s00115-021-01224-8. Online ahead of print.ABSTRACTHuntington disease (HD) is the most frequent monogenetic neurodegenerative disease and can be unequivocally diagnosed even in the preclinical stage, at least in all individuals in whom the CAG expansion mutation in the huntingtin gene (HTT) is in the range of ... read more

Somatic stem cells are essential for maintenance of cell proliferation-differentiation homeostasis in organs. Despite the importance, how the esophageal epithelium that executes its self-renewal and maintenance remains elusive. In this study, using 5-bromo-2'-deoxyuridine (BrdU) label-chase in rat and rat esophageal keratinocyte cell line-derived organoids together with genome-wide DNA methylation profiling ... read more

Trends Neurosci. 2021 Nov 6:S0166-2236(21)00209-5. doi: 10.1016/j.tins.2021.10.009. Online ahead of print.ABSTRACTA recent paper by Elorza et al. describes an 'intersect-RNA-seq' analysis of Huntington's disease (HD) by parallel RNA sequencing (RNA-seq) profiling of HD brain tissues from humans and mice. This work illustrates a broadly applicable strategy to elucidate splicing alterations ... read more

iScience. 2021 Oct 14;24(11):103282. doi: 10.1016/j.isci.2021.103282. eCollection 2021 Nov 19.ABSTRACTHuntington disease (HD) is a devastating neurodegenerative disorder characterized by aggregation of huntingtin (HTT) protein containing expanded polyglutamine (polyQ) tracts. DNAJB6, a member of the DNAJ chaperone family, was reported to efficiently inhibit polyQ aggregation in vitro, in cell models, and ... read more

Crit Rev Anal Chem. 2021 Nov 9:1-20. Online ahead of print.ABSTRACTAs essential neurological chemical messengers, neurotransmitters play an integral role in the maintenance of normal mammalian physiology. Aberrant neurotransmitter activity is associated with a range of neurological conditions including Parkinson's disease, Alzheimer's disease, and Huntington's disease. Many studies to date ... read more

Mov Disord. 2021 Nov 9. doi: 10.1002/mds.28839. Online ahead of print.ABSTRACTBACKGROUND: Huntington's disease is a neurodegenerative disorder characterized by clinical alterations in the motor, behavioral, and cognitive domains. However, the structure and disruptions to large-scale brain cognitive networks have not yet been established.OBJECTIVE: We aimed to profile changes in large-scale ... read more

Front Mol Neurosci. 2021 Oct 22;14:711396. doi: 10.3389/fnmol.2021.711396. eCollection 2021.ABSTRACTWidespread elevations in brain urea have, in recent years, been reported in certain types of age-related dementia, notably Alzheimer's disease (AD) and Huntington's disease (HD). Urea increases in these diseases are substantive, and approximate in magnitude to levels present in uraemic ... read more

Clin Exp Optom. 2021 Nov 9:1-11. doi: 10.1080/08164622.2021.1984179. Online ahead of print.ABSTRACTThe timely detection of neurodegenerative diseases is central to improving clinical care as well as enabling the development and deployment of disease-modifying therapies. Retinal imaging is emerging as a method to detect features of a number of neurodegenerative diseases, ... read more

Am J Nephrol. 2021 Nov 9:1-17. doi: 10.1159/000519811. Online ahead of print.ABSTRACTBACKGROUND: The kynurenine pathway (KP) is the major catabolic pathway for tryptophan degradation. The KP plays an important role as the sole de novo nicotinamide adenine dinucleotide (NAD+) biosynthetic pathway in normal human physiology and functions as a counter-regulatory ... read more

Exp Biol Med (Maywood). 2021 Nov 7:15353702211046927. doi: 10.1177/15353702211046927. Online ahead of print.ABSTRACTHuntington's disease (HD) is a devastating polyglutamine disorder characterized by extensive neurodegeneration and metabolic abnormalities at systemic, cellular and intracellular levels. Metabolic alterations in HD manifest as abnormal body weight, dysregulated biomolecule levels, impaired adipocyte functions, and defective ... read more

J Neurosci Res. 2021 Nov 8. doi: 10.1002/jnr.24977. Online ahead of print.ABSTRACTThe imbalance between glutamate and γ-aminobutyric acid (GABA) results in the loss of synaptic strength leading to neurodegeneration. The dogma on the field considered neurons as the main players in this excitation-inhibition (E/I) balance. However, current strategies focusing only ... read more

Brain Res. 2021 Oct 29;1773:147704. doi: 10.1016/j.brainres.2021.147704. Online ahead of print.ABSTRACTAmong the most studied diseases that affect the central nervous system are Parkinson's, Alzheimer's, and Huntington's diseases, but the lack of effective biomarkers, accurate diagnosis, and precise treatment for each of them is currently an issue. Due to the contribution ... read more

Front Neurol. 2021 Oct 22;12:712565. doi: 10.3389/fneur.2021.712565. eCollection 2021.ABSTRACTVolumetric magnetic resonance imaging (vMRI) has been widely studied in Huntington's disease (HD) and is commonly used to assess treatment effects on brain atrophy in interventional trials. Global and regional trajectories of brain atrophy in HD, with early involvement of striatal regions, ... read more

Neurol India. 2021 Sep-Oct;69(5):1247-1258. doi: 10.4103/0028-3886.329528.ABSTRACTBACKGROUND: Huntington's disease (HD) is a progressive neurodegenerative disorder characterized by motor, cognitive, and psychiatric abnormalities. Currently, matched analyses of structural and functional differences in the brain from the same study cohort and, specifically, in HD patients from an ethnically diverse Indian population are lacking. ... read more

Fac Rev. 2021 Oct 21;10:77. doi: 10.12703/r/10-77. eCollection 2021.ABSTRACTHuntington's disease (HD) is a neurodegenerative disease that results in motor and cognitive dysfunction, leading to early death. HD is caused by an expansion of CAG repeats in the huntingtin gene (HTT). Here, we review the mouse models of HD. They have ... read more

STAR Protoc. 2021 Oct 16;2(4):100886. doi: 10.1016/j.xpro.2021.100886. eCollection 2021 Dec 17.ABSTRACTHuntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by the polyglutamine (polyQ) expansion in huntingtin (HTT) protein. The challenge of obtaining full-length HTT proteins with high purity limits the understanding of the HTT protein function. Here, we provide ... read more

Neuroscience. 2021 Nov 5:S0306-4522(21)00544-3. doi: 10.1016/j.neuroscience.2021.10.025. Online ahead of print.ABSTRACTIn recent decades, our understanding of the molecular changes involved in neurodegenerative diseases has been transformed. Single-cell RNA sequencing and single-nucleus RNA sequencing technologies have been applied to provide cellular and molecular details of the brain at the single-cell level. This ... read more

Biophys Chem. 2021 Nov 1;280:106714. doi: 10.1016/j.bpc.2021.106714. Online ahead of print.ABSTRACTProteins, under certain circumstances such as defective quality control mechanism, mutations and altered environmental conditions, undergo misfolding and assemble into highly ordered beta-sheet structured fibrillar aggregates called amyloid fibrils. Formation of amyloid is seen in most of the protein linked ... read more

Neurotoxicology. 2021 Nov 5:S0161-813X(21)00153-4. doi: 10.1016/j.neuro.2021.11.003. Online ahead of print.ABSTRACTIron is a key element for mitochondrial function and homeostasis, which is also crucial for maintaining the neuronal system, but too much iron promotes oxidative stress. A large body of evidence has indicated that abnormal iron accumulation in the brain is ... read more

Neurodegener Dis. 2021 Nov 8. doi: 10.1159/000520754. Online ahead of print.ABSTRACTIntroduction Autonomic dysfunction has been reported as one of non-motor manifestations of both pre-symptomatic and manifest Huntington's Disease (HD). The aim of our study was to evaluate heart rate variability (HRV) during wake and sleep in a cohort of patients ... read more

Neuroimage Clin. 2021 Oct 27;32:102865. doi: 10.1016/j.nicl.2021.102865. Online ahead of print.ABSTRACTTime processing over intervals of hundreds of milliseconds to minutes, also known as interval timing, is associated with the striatum. Huntington's disease patients (HD) with striatal degeneration have impaired interval timing, but the extent and specificity of these deficits remain ... read more

Neurosci Biobehav Rev. 2021 Nov 3:S0149-7634(21)00489-9. doi: 10.1016/j.neubiorev.2021.10.047. Online ahead of print.ABSTRACTSirtuins, a class III histone/protein deacetylase, is a central regulator of metabolic function and cellular stress response. This plays a pivotal role in the pathogenesis and progression of diseases such as cancer, neurodegeneration, metabolic syndromes, and cardiovascular disease. Sirtuins ... read more

Int J Methods Psychiatr Res. 2021 Nov 5:e1898. doi: 10.1002/mpr.1898. Online ahead of print.ABSTRACTOBJECTIVES: To assess whether prevailing antipsychotic use rates in community nursing homes (CNH) influence new initiation of antipsychotics and diagnosis with antipsychotic indications among Veterans.METHODS: We used linked 2013-2016 Veterans Administration (VA) data, Medicare claims, Nursing Home ... read more

J Biol Chem. 2021 Oct 31:101363. doi: 10.1016/j.jbc.2021.101363. Online ahead of print.ABSTRACTHuntington's disease (HD) is a neurodegenerative disorder caused by a poly-CAG expansion in the first exon of the HTT gene, resulting in an extended poly-glutamine (polyQ) tract in the N-terminal domain of the Huntingtin (Htt) protein product. Proteolytic fragments ... read more

Circ Res. 2021 Sep 3;129(Suppl_1):AMP265. doi: 10.1161/res.129.suppl_1.MP265. Epub 2021 Nov 4.ABSTRACTCyclic nucleotide phosphodiesterases (PDEs), through the degradation of cyclic nucleotides, play critical roles in cardiovascular biology and disease. PDE10A is able to hydrolyze both cAMP and cGMP. Its high expression in medium spiny neurons of the human striatum has led ... read more

J Med Econ. 2021 Nov 3:1-26. doi: 10.1080/13696998.2021.2002579. Online ahead of print.ABSTRACTAIMS: To examine healthcare utilization and costs in a US Medicare population diagnosed with Huntington's disease (HD).METHODS: This was a retrospective matched-cohort study using Medicare fee-for-service (FFS) claims data using 2013-2017 Research Identifiable Files. Medicare beneficiaries diagnosed with HD ... read more

Oxid Med Cell Longev. 2021 Oct 23;2021:5005136. doi: 10.1155/2021/5005136. eCollection 2021.ABSTRACTIncreasing evidence indicates a possible causal link between neuroinflammation and neurological disorders, including Alzheimer's disease (AD), Parkinson's disease (PD), Huntington's disease (HD), and stroke. A putative mechanism underlying such a link can be explained by ferroptosis. Current studies have shown ... read more

Huntington's disease (HD) is a fatal neurodegenerative disorder characterized by progressive motor dysfunction and loss of medium spiny neurons (MSNs) in dorsal striatum. Brain-derived neurotrophic factor (BDNF) sustains functionality and integrity of MSNs, and thus reduced BDNF signaling is integral to the disease. Here we show that SorCS2 is expressed ... read more

J Huntingtons Dis. 2021 Oct 23. doi: 10.3233/JHD-210498. Online ahead of print.ABSTRACTBACKGROUND: The COVID-19 pandemic has increased the need for remote healthcare options among patients with Huntington's disease (HD). However, since not every HD patient is suitable for telehealth, it is important to differentiate who can be seen virtually from ... read more

Curr Alzheimer Res. 2021 Oct 22. doi: 10.2174/1567205018666211022164025. Online ahead of print.ABSTRACTNitric oxide synthase (NOS) is well known for its involvement in the regulation of the nervous, cardiovascular, and immune systems. Neuronal NOS (nNOS) is the most characterized NOS among all the isoforms. It accounts for most of the production ... read more

J Huntingtons Dis. 2021 Oct 23. doi: 10.3233/JHD-210493. Online ahead of print.ABSTRACTBACKGROUND: Falls are common in Huntington's disease (HD), which can have serious consequences and may therefore lead to fear of falling (FoF). There is little knowledge about falls or FoF in individuals with HD or about formal and informal ... read more

J Huntingtons Dis. 2021 Oct 25. doi: 10.3233/JHD-210497. Online ahead of print.ABSTRACTOlfactory dysfunction is a common symptom in patients with neurodegenerative disorders, including Huntington's disease (HD). Understanding its pathophysiology is important in establishing a preventive and therapeutic plan. In this literature review, we cover the physiology of olfaction, its role ... read more

J Clin Exp Immunol. 2021;6(5):367-372. doi: 10.33140/jcei.06.05.04. Epub 2021 Oct 20.ABSTRACTCinnamon is a regularly used natural seasoning and flavoring material throughout the world for eras. Recent laboratory studies have demonstrated that oral cinnamon may be beneficial for different neuroinflammatory and neurodegenerative disorders such as multiple sclerosis (MS), Parkinson's disease (PD), ... read more

Front Genet. 2021 Oct 15;12:751033. doi: 10.3389/fgene.2021.751033. eCollection 2021.ABSTRACTHuntington's disease (HD) is a chronic neurodegenerative disorder caused by an expansion of polyglutamine repeats in exon 1 of the Huntingtin gene. Transcriptional dysregulation accompanied by epigenetic alterations is an early and central disease mechanism in HD yet, the exact mechanisms and ... read more

Front Mol Neurosci. 2021 Oct 15;14:721749. doi: 10.3389/fnmol.2021.721749. eCollection 2021.ABSTRACTHuntington's disease is a dominantly inherited neurodegenerative disorder caused by the expansion of a CAG repeat, encoding for the amino acid glutamine (Q), present in the first exon of the protein huntingtin. Over the threshold of Q39 HTT exon 1 (HTTEx1) ... read more

J Cell Physiol. 2021 Nov 1. doi: 10.1002/jcp.30624. Online ahead of print.ABSTRACTNoncoding RNAs (ncRNAs) are important regulators of gene expression in different cell processes. Due to their ability in monitoring neural development genes, these transcripts confer neurons with the potential to exert broad control over the expression of genes for ... read more

Nucleic Acids Res. 2021 Oct 28:gkab898. doi: 10.1093/nar/gkab898. Online ahead of print.ABSTRACTIn neurodegenerative diseases, including pathologies with well-known causative alleles, genetic factors that modify severity or age of onset are not entirely understood. We recently documented the unexpected prevalence of transfer RNA (tRNA) mutants in the human population, including variants ... read more

Nucleic Acids Res. 2021 Oct 28:gkab899. doi: 10.1093/nar/gkab899. Online ahead of print.ABSTRACTThe Repeat Expansion Diseases, a large group of human diseases that includes the fragile X-related disorders (FXDs) and Huntington's disease (HD), all result from expansion of a disease-specific microsatellite via a mechanism that is not fully understood. We have ... read more