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Oncol Lett. 2021 Jul;22(1):564. doi: 10.3892/ol.2021.12825. Epub 2021 May 29.ABSTRACTThe study of the disorders of ubiquitin-mediated proteasomal degradation may unravel the molecular basis of human diseases, such as cancer (prostate cancer, lung cancer and liver cancer, etc.) and nervous system disease (Parkinson's disease, Alzheimer's disease and Huntington's disease, etc.) and ... read more

Nat Biotechnol. 2021 Jun;39(6):650-652. doi: 10.1038/s41587-021-00955-y.NO ABSTRACTPMID:34113039 | DOI:10.1038/s41587-021-00955-y ... read more

PLoS One. 2021 Jun 11;16(6):e0252683. doi: 10.1371/journal.pone.0252683. eCollection 2021.ABSTRACTBACKGROUND: Huntington's disease (HD) is an inherited, progressive neurodegenerative disease that has no cure. Striatal atrophy and hypometabolism has been described in HD as far as 15 years before clinical onset and therefore structural and functional imaging biomarkers are the most applied ... read more

Front Pharmacol. 2021 May 24;12:656561. doi: 10.3389/fphar.2021.656561. eCollection 2021.ABSTRACTEffective treatments for neurodegenerative diseases remain elusive and are critically needed since the burden of these diseases increases across an aging global population. Nitric oxide (NO) is a gasotransmitter that binds to soluble guanylate cyclase (sGC) to produce cyclic guanosine monophosphate (cGMP). ... read more

PLoS One. 2021 Jun 10;16(6):e0253037. doi: 10.1371/journal.pone.0253037. eCollection 2021.ABSTRACTDescription of robust transcriptomic alterations in Huntington's disease is essential to identify targets for biochemical studies and drug development. We analysed publicly available transcriptome data from the brain and blood of 220 HD patients and 241 healthy controls and identified 737 and ... read more

Front Neuroanat. 2021 May 24;15:673177. doi: 10.3389/fnana.2021.673177. eCollection 2021.ABSTRACTHuntington's disease (HD) is a dominantly inherited, adult-onset neurodegenerative disease characterized by motor, psychiatric, and cognitive abnormalities. Neurodegeneration is prominently observed in the striatum where GABAergic medium spiny neurons (MSN) are the most affected neuronal population. Interestingly, recent reports of pathological changes ... read more

Brain Behav. 2021 Jun 10:e02151. doi: 10.1002/brb3.2151. Online ahead of print.ABSTRACTINTRODUCTION: Behavioral and cognitive changes can be observed across all Huntington disease (HD) stages. Our multicenter and retrospective study investigated the association between cognitive and behavioral scale scores in manifest HD, at three different yearly timepoints.METHODS: We analyzed cognitive and ... read more

Biochem Pharmacol. 2021 Jun 7:114650. doi: 10.1016/j.bcp.2021.114650. Online ahead of print.ABSTRACTParkin is a cytosolic E3 ubiquitin ligase that plays an important role in neuroprotection by targeting several proteins to be degraded by the 26S proteasome. Its dysfunction has been associated not only with Parkinson's disease (PD) but also with other ... read more

Neurochem Int. 2021 Jun 7:105095. doi: 10.1016/j.neuint.2021.105095. Online ahead of print.ABSTRACTMitochondria are semi-autonomous organelle staging a crucial role in cellular stress response, energy metabolism and cell survival. Maintaining mitochondrial quality control is very important for its homeostasis. Pathological conditions such as oxidative stress and neurodegeneration, disrupt this quality control, and ... read more

The default mode network (DMN) is the most prominent intrinsic connectivity network, serving as a key architecture of the brain's functional organization. Conversely, dysregulation of the DMN is characteristic of major neuropsychiatric disorders. However, the field still lacks mechanistic insights into the regulation of the DMN and effective interventions for ... read more

Curr Neuropharmacol. 2021 Jun 8. doi: 10.2174/1570159X19666210608165509. Online ahead of print.ABSTRACTNeurological disorders like Alzheimer's disease (AD), Parkinson's disease (PD), stroke, amyotrophic lateral sclerosis, Huntington's disease (HD), epilepsy, traumatic brain injury (TBI), depression and anxiety are responsible for thousands of deaths worldwide every year. With the increase in life expectancy, there ... read more

Monoamine transporters retrieve serotonin (SERT), dopamine (DAT) and norepinephrine (NET) from the synaptic cleft. Surface levels of transporters are also regulated by internalization. Clathrin-mediated endocytosis of cargo proteins requires adaptor protein 2 (AP2), which recruits cargo to the nascent clathrin-cage. The transporter C-terminus is required for internalization but lacks an ... read more

Cell Death Differ. 2021 Jun 7. doi: 10.1038/s41418-021-00814-y. Online ahead of print.ABSTRACTTightly orchestrated programmed cell death (PCD) signalling events occur during normal neuronal development in a spatially and temporally restricted manner to establish the neural architecture and shaping the CNS. Abnormalities in PCD signalling cascades, such as apoptosis, necroptosis, pyroptosis, ... read more

Front Neurol. 2021 May 20;12:678484. doi: 10.3389/fneur.2021.678484. eCollection 2021.ABSTRACTHuntington's disease (HD) is characterised by a triad of cognitive, behavioural, and motor symptoms which lead to functional decline and loss of independence. With potential disease-modifying therapies in development, there is interest in accurately measuring HD progression and characterising prognostic variables to ... read more

J Huntingtons Dis. 2021 Jun 4. doi: 10.3233/JHD-210467. Online ahead of print.ABSTRACTThe role of oxytocin (OT) in social cognition of patients with Huntington's disease (HD) has been studied, but its impact on executive functioning has not been explored yet. Healthy controls, premanifest HD, and manifest HD participants underwent executive functioning ... read more

J Huntingtons Dis. 2021 Jun 4. doi: 10.3233/JHD-200463. Online ahead of print.ABSTRACTBACKGROUND: Therapeutics that lower mutant huntingtin (mHTT) have shown promise in preclinical studies and are in clinical development for the treatment of Huntington's disease (HD). Multiple assays have been developed that either quantify mHTT or total HTT but may ... read more

Ageing Res Rev. 2021 Jun 4:101385. doi: 10.1016/j.arr.2021.101385. Online ahead of print.ABSTRACTHuntington's disease (HD) is an autosomal neurodegenerative disorder caused by extended trinucleotide CAG repetition in the HTT gene. Wild-type huntingtin protein (HTT) is essential, involved in a variety of crucial cellular functions such as vesicle transportation, cell division, transcription ... read more

Neurologia. 2021 Jun 2:S0213-4853(21)00090-6. doi: 10.1016/j.nrl.2021.04.016. Online ahead of print.ABSTRACTOBJECTIVE: To identify the neurological diseases for which euthanasia and assisted suicide are most frequently requested in the countries where these medical procedures are legal and the specific characteristics of euthanasia in some of these diseases, and to show the evolution ... read more

Neurologia. 2021 Jun 2:S0213-4853(21)00087-6. doi: 10.1016/j.nrl.2021.04.015. Online ahead of print.ABSTRACTINTRODUCTION: Huntington disease (HD) is a hereditary neurodegenerative disorder. Thanks to predictive diagnosis, incipient clinical characteristics have been described in the prodromal phase.OBJECTIVE: To compare performance in cognitive tasks of carriers (HDC) and non-carriers (non-HDC) of the huntingtin gene and to ... read more

Neuroscientist. 2021 Jun 4:10738584211021018. doi: 10.1177/10738584211021018. Online ahead of print.ABSTRACTMetabotropic glutamate receptors (mGluRs) are G-protein coupled receptors that are activated by glutamate in the central nervous system (CNS). Basically, mGluRs contribute to fine-tuning of synaptic efficacy and control the accuracy and sharpness of neurotransmission. Among eight subtypes, mGluR1 and mGluR5 ... read more

Biochim Biophys Acta Biomembr. 2021 Jun 2:183663. doi: 10.1016/j.bbamem.2021.183663. Online ahead of print.ABSTRACTHuntington's disease (HD) is a neurodegenerative disease caused by the expansion of a polyglutamine (polyQ) tract near the N-terminus of the huntingtin (htt) protein. Expanded polyQ tracts are prone to aggregate into oligomers and insoluble fibrils. Mutant htt ... read more

Pilot Feasibility Stud. 2021 Jun 3;7(1):119. doi: 10.1186/s40814-021-00833-z.ABSTRACTOBJECTIVE: It is unclear whether how people with long-term conditions express distress, and how clinicians respond, influences perceptions of consultation outcomes. The pilot study examined emotional distress and reassurance in consultations with people whose long-term conditions (at the time of consultations) were treated ... read more

PLoS One. 2021 Jun 4;16(6):e0252380. doi: 10.1371/journal.pone.0252380. eCollection 2021.ABSTRACTThis study proposes a reliable computer-aided framework to identify gait fluctuations associated with a wide range of degenerative neuromuscular disease (DNDs) and health conditions. Investigated DNDs included amyotrophic lateral sclerosis (ALS), Parkinson's disease (PD), and Huntington's disease (HD). We further performed a ... read more

Prog Neuropsychopharmacol Biol Psychiatry. 2021 Jun 1:110368. doi: 10.1016/j.pnpbp.2021.110368. Online ahead of print.ABSTRACTHuntington's disease (HD) is characterized by abnormal involuntary movements together with cognitive impairment and disrupted mood changes. 3-nitropropionic acid (3-NP) is one of the chemo-toxic models used to address the striatal neurotoxicity pattern encountered in HD. This study ... read more

Technological advances, lack of medical professionals, high cost of face-to face encounters and disasters such as COVID19 pandemic, fuel the telemedicine revolution. Numerous smartphone apps have been developed to measure neurological functions. However, their psychometric properties are seldom determined. Lacking such data, it is unclear which designs underlie eventual clinical ... read more

A loss of the checkpoint kinase ATM leads to impairments in the DNA damage response, and in humans causes cerebellar neurodegeneration, and a high risk to cancer. A loss of ATM is also associated with increased protein aggregation. The relevance and characteristics of this aggregation are still incompletely understood. Moreover, ... read more

Brain Sci. 2021 May 27;11(6):710. doi: 10.3390/brainsci11060710.ABSTRACTBACKGROUND: There is a broad range of potential differential diagnoses for chorea. Besides rare, inherited neurodegenerative diseases such as Huntington's disease (HD) chorea can accompany basal ganglia disorders due to vasculitis or infections, e.g., with the human immunodeficiency virus (HIV). The clinical picture is ... read more

Int J Mol Sci. 2021 May 31;22(11):5911. doi: 10.3390/ijms22115911.ABSTRACTAlzheimer's disease (AD) is a mostly sporadic brain disorder characterized by cognitive decline resulting from selective neurodegeneration in the hippocampus and cerebral cortex whereas Huntington's disease (HD) is a monogenic inherited disorder characterized by motor abnormalities and psychiatric disturbances resulting from selective ... read more

Genes (Basel). 2021 May 10;12(5):712. doi: 10.3390/genes12050712.ABSTRACTWhite matter atrophy has been shown to precede the massive loss of striatal GABAergic neurons in Huntington's disease (HD). This study investigated the effects of in vivo expression of reprogramming factor octamer-binding transcription factor 4 (OCT4) on neural stem cell (NSC) niche activation in ... read more

Int J Mol Sci. 2021 May 31;22(11):5887. doi: 10.3390/ijms22115887.ABSTRACTGlucose is the main circulating energy substrate for the adult brain. Owing to the high energy demand of nerve cells, glucose is actively oxidized to produce ATP and has a synergistic effect with mitochondria in metabolic pathways. The dysfunction of glucose metabolism ... read more

Int J Environ Res Public Health. 2021 May 12;18(10):5113. doi: 10.3390/ijerph18105113.ABSTRACTHuntington's disease (HD) is a neurodegenerative dementia with a well recognised genetic cause. Alcohol misuse is a major environmental factor relevant to numerous neurological presentations, including HD. We explored the effects of alcohol intake on clinical features of HD by ... read more

Int J Mol Sci. 2021 May 18;22(10):5326. doi: 10.3390/ijms22105326.ABSTRACTRhes is one of the most interesting genes regulated by thyroid hormones that, through the inhibition of the striatal cAMP/PKA pathway, acts as a modulator of dopamine neurotransmission. Rhes mRNA is expressed at high levels in the dorsal striatum, with a medial-to-lateral ... read more

Int J Mol Sci. 2021 May 28;22(11):5795. doi: 10.3390/ijms22115795.ABSTRACTNeurodegenerative diseases (NDs), such as Alzheimer's disease (AD), Parkinson's disease (PD), Huntington's disease (HD), and amyotrophic lateral sclerosis (ALS), are disorders characterized by progressive degeneration of the nervous system. Currently, there is no disease-modifying treatments for most NDs. Meanwhile, numerous studies conducted ... read more

Molecules. 2021 May 31;26(11):3314. doi: 10.3390/molecules26113314.ABSTRACTThe pathogenesis of several neurodegenerative diseases such as Alzheimer's or Huntington's disease has been associated with metabolic dysfunctions caused by imbalances in the brain and cerebral spinal fluid levels of neuroactive metabolites. Kynurenine monooxygenase (KMO) is considered an ideal therapeutic target for the regulation of ... read more

Int J Mol Sci. 2021 May 18;22(10):5292. doi: 10.3390/ijms22105292.ABSTRACTThe number of patients with neurodegenerative diseases (NDs) is increasing, along with the growing number of older adults. This escalation threatens to create a medical and social crisis. NDs include a large spectrum of heterogeneous and multifactorial pathologies, such as amyotrophic lateral ... read more

Sensors (Basel). 2021 May 31;21(11):3825. doi: 10.3390/s21113825.ABSTRACTHuntington's disease (HD) is an autosomal dominant, incurable neurodegenerative disease caused by mutation in the huntingtin gene (HTT). HTT mutation leads to protein misfolding and aggregation, which affect cells' functions and structural features. Because these changes might modify the scattering strength of affected cells, ... read more

Int J Mol Sci. 2021 May 21;22(11):5444. doi: 10.3390/ijms22115444.ABSTRACTC60 fullerene as a potent free radical scavenger and antioxidant could be a beneficial means for neurodegenerative disease prevention or cure. The aim of the study was to define the effects of C60 administration on mitochondrial dysfunction and oxidative stress disorders in ... read more

BMC Nephrol. 2021 Jun 1;22(1):204. doi: 10.1186/s12882-021-02417-8.ABSTRACTBACKGROUNDS: Previous studies have demonstrated that excretion of urinary extracellular vesicles (EVs) from different nephron segments differs between kidney stone formers and non-stone formers (NSFs), and could reflect pathogenic mechanisms of urinary stone disease. In this study we quantified selected populations of specific urinary ... read more

Int J Mol Sci. 2021 May 12;22(10):5116. doi: 10.3390/ijms22105116.ABSTRACTThe multiple functions of the wild type Huntington's disease protein of the sea urchin Hemicentrotus pulcherrimus (Hp-Htt) have been examined using the anti-Hp-Htt antibody (Ab) raised against synthetic oligopeptides. According to immunoblotting, Hp-Htt was detected as a single band at around the ... read more

Eur J Neurol. 2021 Jun 2. doi: 10.1111/ene.14953. Online ahead of print.ABSTRACTBACKGROUND: Dysphagia is one of the most common and important complications in Huntington's Disease (HD), frequently leading to aspiration pneumonia and mortality. Objective estimates of prevalence using instrumental diagnostics and data on neural correlates of dysphagia in HD are ... read more

Cognition. 2021 May 29:104785. doi: 10.1016/j.cognition.2021.104785. Online ahead of print.ABSTRACTMore than a century ago, Broca (1861), Wernicke (1874) and Lichteim (1885) laid the foundations for the first anatomo-functional model of language, secondarily enriched by Geschwind (1967), leading to the Broca-Wernicke-Lichteim-Geschwind model. This model included the frontal, parietal, and temporal cortices ... read more

Naunyn Schmiedebergs Arch Pharmacol. 2021 Jun 1. doi: 10.1007/s00210-021-02106-1. Online ahead of print.ABSTRACTMitochondrial abnormalities and redox imbalance are major pathogenic factors in progression of Huntington's disease (HD), manifested clinically by affective, motor, cognitive, and psychiatric incompetence. Antioxidants behold much promise in mitigation of several pathological facets in HD. Ellagic acid ... read more

Front Cell Dev Biol. 2021 May 13;9:635104. doi: 10.3389/fcell.2021.635104. eCollection 2021.ABSTRACTExtracellular vesicles (EVs) are nano-sized membrane-enclosed particles released by cells that participate in intercellular communication through the transfer of biologic material. EVs include exosomes that are small vesicles that were initially associated with the disposal of cellular garbage; however, recent ... read more

Front Pharmacol. 2021 May 14;12:672182. doi: 10.3389/fphar.2021.672182. eCollection 2021.ABSTRACTAdenosine, acting both through G-protein coupled adenosine receptors and intracellularly, plays a complex role in multiple physiological and pathophysiological processes by modulating neuronal plasticity, astrocytic activity, learning and memory, motor function, feeding, control of sleep and aging. Adenosine is involved in stroke, ... read more

Neural Plast. 2021 May 16;2021:8884642. doi: 10.1155/2021/8884642. eCollection 2021.ABSTRACTNeurodegenerative disorders (NDs) are characterized by a gradual loss of neurons and functions that eventually leads to progressive neurological impairment. In view of the heavy burden on the healthcare system, efficient and reliable biomarkers for early diagnosis and therapeutic treatments to reverse ... read more

Front Neurol. 2021 May 13;12:658123. doi: 10.3389/fneur.2021.658123. eCollection 2021.ABSTRACTBackground: Huntington's disease (HD) is a progressive neurodegenerative disorder. Generally, it is characterized by deficits in cognition, behavior, and movement. Recent studies have shown that pridopidine is a potential and effective drug candidate for the treatment of HD. In the present study, ... read more

Neurosci Biobehav Rev. 2021 May 28;127:709-739. doi: 10.1016/j.neubiorev.2021.05.021. Online ahead of print.ABSTRACTSelf and proxy reported questionnaires indicate that people living with a neurodegenerative disease (NDD) have more difficulties with financial decision-making (FDM) than healthy controls. Self-reports, however, rely on adequate insight into everyday functioning and might, therefore, be less reliable. ... read more

Brain Res. 2021 May 27:147540. doi: 10.1016/j.brainres.2021.147540. Online ahead of print.ABSTRACTMutation in proteins containing polyglutamine (polyQ) tracts has been shown to underlie a number of severe human neurodegenerative disorders such as Huntington's Disease and Spinocerebellar Ataxia. In this study, we identify and describe FAM171B as a novel polyQ protein containing ... read more

Nat Commun. 2021 May 28;12(1):3216. doi: 10.1038/s41467-021-23491-4.ABSTRACTX-linked dystonia-parkinsonism is a neurodegenerative disorder caused by a founder retrotransposon insertion, in which a polymorphic hexanucleotide repeat accounts for ~50% of age at onset variability. Employing a genome-wide association study to identify additional factors modifying age at onset, we establish that three independent ... read more

Microsc Res Tech. 2021 May 28. doi: 10.1002/jemt.23842. Online ahead of print.ABSTRACTN-Nitrosodibutylamine (DBN) has been found in a wide variety of products because of the nitrosation of amines present in these products. An extensive series of synthesis and carcinogenicity studies of various compounds related to DBN and its metabolites have ... read more

Parkinsonism Relat Disord. 2021 May 20;88:1-2. doi: 10.1016/j.parkreldis.2021.05.012. Online ahead of print.ABSTRACTBACKGROUND: No study investigated the prevalence of pediatric Huntington's Disease.METHODS: Prevalence was analyzed in ENROLL-HD from participants and family trees.RESULTS: 50 compared with 7544 adult (0.66%) and 22 out of 16,124 family participants (0.14%) were pediatric.CONCLUSIONS: The pediatric prevalence ... read more

CNS Neurol Disord Drug Targets. 2021 May 26. doi: 10.2174/1871527320666210526160214. Online ahead of print.ABSTRACTAMP-activated protein kinase (AMPK) is a serine/threonine kinase and a driving or deterrent factor in the development of neurodegenerative diseases and dementia. AMPK affects intracellular proteins like the mammalian target of rapamycin (mTOR). Peroxisome proliferator-activated receptor-γ coactivator ... read more

Brain. 2021 May 27:awab190. doi: 10.1093/brain/awab190. Online ahead of print.ABSTRACTHuntington's disease is a dominantly inherited, fatal neurodegenerative disorder caused by a CAG expansion in the Huntingtin (HTT) gene, coding for pathologic mutant HTT protein (mHTT). Because of its gain-of-function mechanism and monogenic etiology, strategies to lower HTT are being actively ... read more

Chicken long-range linkage disequilibrium (LRLD) and LD blocks, and their relationship with previously described Mareks Disease (MD) quantitative trait loci regions (QTLRs), were studied in an F6 population from a full-sib advanced intercross line (FSAIL), and in eight commercial pure layer lines. Genome wide LRLD was studied in the F6 ... read more

Alterations in the serotonergic control of brain pathways responsible for facial-emotion processing in people with autism spectrum disorder (ASD) may be a target for intervention. However, the molecular underpinnings of autistic-neurotypical serotonergic differences are challenging to access in vivo. Receptor-Enriched Analysis of functional Connectivity by Targets (REACT) has helped define ... read more

Acta Neuropathol Commun. 2021 May 25;9(1):98. doi: 10.1186/s40478-021-01201-x.ABSTRACTBACKGROUND: Short tandem repeat (STR) expansion disorders are an important cause of human neurological disease. They have an established role in more than 40 different phenotypes including the myotonic dystrophies, Fragile X syndrome, Huntington's disease, the hereditary cerebellar ataxias, amyotrophic lateral sclerosis and ... read more

Neurochem Int. 2021 May 23:105074. doi: 10.1016/j.neuint.2021.105074. Online ahead of print.ABSTRACTHuntington's disease (HD) is a devastating and fatal monogenic neurodegenerative disorder characterized by progressive loss of selective neurons in the brain and is caused by an abnormal expansion of CAG trinucleotide repeats in a coding exon of the huntingtin (HTT) ... read more

J Neurosci Res. 2021 May 24. doi: 10.1002/jnr.24825. Online ahead of print.ABSTRACTExtensive alterations in gene regulatory networks are a typical characteristic of Huntington's disease (HD); these include alterations in protein-coding genes and poorly understood non-coding RNAs (ncRNAs), which are associated with pathology caused by mutant huntingtin. Long non-coding RNAs (lncRNAs) ... read more

Autophagy. 2021 May 24:1-22. doi: 10.1080/15548627.2021.1904489. Online ahead of print.ABSTRACTCurrent disease-modifying therapies for Huntington disease (HD) focus on lowering mutant HTT (huntingtin; mHTT) levels, and the immunosuppressant drug rapamycin is an intriguing therapeutic for aging and neurological disorders. Rapamycin interacts with FKBP1A/FKBP12 and FKBP5/FKBP51, inhibiting the MTORC1 complex and increasing ... read more

J Appl Stat. 2021;48(7):1339-1348. doi: 10.1080/02664763.2020.1759034. Epub 2020 Apr 27.ABSTRACTWhile there is no known cure for Huntington's disease (HD), there are early-phase clinical trials aimed at altering disease progression patterns. There is, however, no obvious single outcome for these trials to evaluate treatment efficacy. Currently used outcomes are, while reasonable, ... read more

Stem Cells. 2021 May 24. doi: 10.1002/stem.3420. Online ahead of print.ABSTRACTHuntington's disease (HD) is an autosomal dominant neurodegenerative disorder characterised by the progressive loss of striatal medium spiny neurons. Using a highly efficient protocol for direct reprogramming of adult human fibroblasts with chemically modified mRNA, we report the first generation ... read more

Front Neurol. 2021 May 5;12:657973. doi: 10.3389/fneur.2021.657973. eCollection 2021.ABSTRACTThe momentum of gene therapy in Huntington's disease (HD) deserves biomarkers from easily accessible fluid. We planned a study to verify whether plasma miRNome may provide useful peripheral "reporter(s)" for the management of HD patients. We performed an exploratory microarray study of ... read more

Ageing Res Rev. 2021 May 20:101367. doi: 10.1016/j.arr.2021.101367. Online ahead of print.ABSTRACTNeurodegenerative diseases are one of the most common diseases in mankind. Although there are reports of several candidates that cause neurodegenerative diseases, the exact mechanism of pathogenesis is poorly understood. The ubiquitin-proteasome system (UPS) is an important posttranslational modification ... read more

Neurochem Int. 2021 May 19:105068. doi: 10.1016/j.neuint.2021.105068. Online ahead of print.ABSTRACT7,8-dihydroxyflavone (DHF), a naturally-occurring plant-based flavone, is a high-affinity tyrosine kinase receptor B (TrkB) agonist, and a bioactive molecule of therapeutic interest for neuronal survival, differentiation, synaptic plasticity and neurogenesis. In the family of neurotrophic factors, this small BDNF-mimetic molecule ... read more

BMJ Case Rep. 2021 May 19;14(5):e241929. doi: 10.1136/bcr-2021-241929.ABSTRACTA functionally independent man in his 20s with a history of intellectual disability and epilepsy and family history of Huntington's disease suffered a severe traumatic brain injury. Postinjury, bilateral chorea rendered him dependent for all activities of daily living. Risperidone provided a significant ... read more

J Neurosci. 2021 May 13:JN-RM-2074-20. doi: 10.1523/JNEUROSCI.2074-20.2021. Online ahead of print.ABSTRACTHuntington's disease (HD) is a dominantly inherited neurodegenerative disorder caused by a trinucleotide expansion in exon 1 of the huntingtin (HTT) gene. Cell death in HD occurs primarily in striatal medium spiny neurons (MSNs), but the involvement of specific MSN ... read more

Eur J Neurosci. 2021 May 20. doi: 10.1111/ejn.15316. Online ahead of print.ABSTRACTAlzheimer's, Parkinson's, and Huntington's disease are characterized by selective degeneration of specific brain areas. Although increasing number of studies report alteration of the extracellular matrix on these diseases, an exhaustive characterization at the brain's matrix level might contribute to ... read more

Evid Based Complement Alternat Med. 2021 Apr 30;2021:5521519. doi: 10.1155/2021/5521519. eCollection 2021.ABSTRACTAs innate immune effector cells in the central nervous system (CNS), microglia not only are essential for the normal development of nervous system but also act on different neurological diseases, including Alzheimer's disease (AD), Huntington's disease (HD), and other ... read more

Mov Disord. 2021 May 20. doi: 10.1002/mds.28650. Online ahead of print.ABSTRACTBACKGROUND: Potential therapeutic targets and clinical trials for Huntington's disease have grown immensely in the last decade. However, to improve clinical trial outcomes, there is a need to better characterize profiles of signs and symptoms across different epochs of the ... read more

Nat Rev Drug Discov. 2021 May 19. doi: 10.1038/d41573-021-00088-6. Online ahead of print.NO ABSTRACTPMID:34012000 | DOI:10.1038/d41573-021-00088-6 ... read more

J Drug Deliv Sci Technol. 2021 Jun;63:102517. doi: 10.1016/j.jddst.2021.102517. Epub 2021 Apr 6.ABSTRACTThis report describes the kinetics of Huntington's Disease (HD) gene (HTT) lowering in brains of YAC 128 mice. Lowering (or "knock-down") of HTT mRNA expression was achieved by intranasal administration of specially designed siRNA loaded into chitosan nanoparticles. ... read more

Bioorg Med Chem Lett. 2021 May 17;44:128115. doi: 10.1016/j.bmcl.2021.128115. Online ahead of print.ABSTRACTKynurenine monooxygenase (KMO) is expected to be a good drug target to treat Huntington's disease (HD). This study presents the structure-activity relationship of pyridazine derivatives to find novel KMO inhibitors. The most promising compound 14 resolved the problematic ... read more

J Med Chem. 2021 May 17. doi: 10.1021/acs.jmedchem.1c00325. Online ahead of print.ABSTRACTPyroglutamate (pE) modification, catalyzed mainly by glutaminyl cyclase (QC), is prevalent throughout nature and is particularly important in mammals including humans for the maturation of hormones, peptides, and proteins. In humans, the upregulation of QC is involved in multiple ... read more

Neurology. 2021 May 18;96(20):940-943. doi: 10.1212/WNL.0000000000011944.NO ABSTRACTPMID:34001541 | DOI:10.1212/WNL.0000000000011944 ... read more

Front Pharmacol. 2021 Apr 28;12:653842. doi: 10.3389/fphar.2021.653842. eCollection 2021.ABSTRACTGangliosides are major constituents of the plasma membrane and are known to promote a number of physiological actions in the brain, including synaptic plasticity and neuroprotection. In particular, the ganglioside GM1 was found to have a wide range of preclinical and clinical ... read more

Front Neurosci. 2021 Apr 28;15:662242. doi: 10.3389/fnins.2021.662242. eCollection 2021.ABSTRACTAlzheimer's disease (AD) is the most common form of age-related dementia. Despite decades of research, the etiology and pathogenesis of AD are not well understood. Brain glucose hypometabolism has long been recognized as a prominent anomaly that occurs in the preclinical stage ... read more

Front Aging Neurosci. 2021 Apr 28;13:638208. doi: 10.3389/fnagi.2021.638208. eCollection 2021.ABSTRACTThere is a growing evidence describing a decline in adaptive homeostasis in aging-related diseases affecting the central nervous system (CNS), many of which are characterized by the appearance of non-native protein aggregates. One signaling pathway that allows cell adaptation is the ... read more

Mov Disord. 2021 May 17. doi: 10.1002/mds.28638. Online ahead of print.ABSTRACTBACKGROUND: Apathy, a common neuropsychiatric disturbance in Huntington's disease (HD), is subserved by a complex neurobiological network. However, no study has yet employed a whole-brain approach to examine underlying regional vulnerabilities that may precipitate apathy changes over time.OBJECTIVES: To identify ... read more

J Huntingtons Dis. 2021 May 14. doi: 10.3233/JHD-200465. Online ahead of print.ABSTRACTMass spectrometry (MS) is a physical technique used to identify specific chemicals and molecules by precise analysis of their mass and charge; this technology has been adapted for biological sciences applications. Investigators have used MS to identify differential expressions ... read more

Prog Neuropsychopharmacol Biol Psychiatry. 2021 May 12:110349. doi: 10.1016/j.pnpbp.2021.110349. Online ahead of print.ABSTRACTThere is currently no effective treatment either for neurological illnesses (ischemia and neurodegenerative diseases) or psychiatric disorders (depression), in which the Glu/GABA balance is disturbed and accompanied by significant excitotoxicity. Therefore, the search for new and effective therapeutic ... read more

Phytomedicine. 2021 Apr 18:153576. doi: 10.1016/j.phymed.2021.153576. Online ahead of print.ABSTRACTBACKGROUND: Gastrodiae Rhizoma (Tianma), the dried tuber of Gastrodia elata Bl. (Orchidaceae), is listed as a top-grade herbal medicine in Shen-nong Ben-ts'ao Jing and has been used for treating headaches, dizziness, vertigo and convulsion. It has a neuroprotective effect and extends ... read more

Exp Ther Med. 2021 Jul;22(1):697. doi: 10.3892/etm.2021.10129. Epub 2021 May 2.ABSTRACTMalignant tumors are often exposed to hypoxic and glucose-starved microenvironments. AMP-activated protein kinase (AMPK) is an energy sensor that is stimulated during energy-deficient conditions and protects cells from hypoxic injury by regulating metabolism. AMPK-related protein kinase 5 (ARK5) is a ... read more

PLoS One. 2021 May 14;16(5):e0250606. doi: 10.1371/journal.pone.0250606. eCollection 2021.ABSTRACTHuntington's disease (HD) is a neurodegenerative disorder caused by a dominant CAG-repeat expansion in the huntingtin gene. Microglial activation is a key feature of HD pathology, and is present before clinical disease onset. The kynurenine pathway (KP) of tryptophan degradation is activated ... read more

Mov Disord Clin Pract. 2021 Mar 30;8(4):555-562. doi: 10.1002/mdc3.13195. eCollection 2021 May.ABSTRACTBACKGROUND: Huntington disease (HD) is an inherited neurodegenerative disorder characterized by motor, psychiatric, and cognitive symptoms. Little is known about the effects of environmental factors on HD symptom onset and severity.OBJECTIVE: To evaluate the relationship between education level and ... read more

Aust N Z J Psychiatry. 2021 May 13:48674211013082. doi: 10.1177/00048674211013082. Online ahead of print.NO ABSTRACTPMID:33982621 | DOI:10.1177/00048674211013082 ... read more

Mov Disord Clin Pract. 2021 Mar 9;8(4):521-524. doi: 10.1002/mdc3.13180. eCollection 2021 May.ABSTRACTElectroconvulsive therapy (ECT) has been a very well known therapy in Psychiatry for over 80 years. ECT is considered useful in treating acute mania, severe depression and other psychiatric conditions. Over time, this therapy has also been used in ... read more

Elife. 2021 May 13;10:e64674. doi: 10.7554/eLife.64674.ABSTRACTRecent work on Huntington disease (HD) suggests that somatic instability of CAG repeat tracts, which can expand into the hundreds in neurons, explains clinical outcomes better than the length of the inherited allele. Here, we measured somatic expansion in blood samples collected from the same ... read more

Exp Neurol. 2021 May 10:113755. doi: 10.1016/j.expneurol.2021.113755. Online ahead of print.ABSTRACTBACKGROUND: RTP801/REDD1 is a stress-regulated protein whose upregulation is necessary and sufficient to trigger neuronal death in in vitro and in vivo models of Parkinson's and Huntington's diseases and is up regulated in compromised neurons in human postmortem brains of ... read more

PLoS One. 2021 May 13;16(5):e0251767. doi: 10.1371/journal.pone.0251767. eCollection 2021.ABSTRACTSleep disturbance is a common and disruptive symptom of neurodegenerative diseases such as Alzheimer's and Huntington's disease (HD). In HD patients, sleep fragmentation appears at an early stage of disease, although features of the earliest sleep abnormalities in presymptomatic HD are not ... read more

PLoS Genet. 2021 May 13;17(5):e1009558. doi: 10.1371/journal.pgen.1009558. eCollection 2021 May.ABSTRACTPolyglutamine diseases are neurodegenerative diseases caused by the expansion of polyglutamine (polyQ) tracts within different proteins. Although multiple pathways have been found to modulate aggregation of the expanded polyQ proteins, the mechanisms by which polyQ tracts induced neuronal cell death remain ... read more

Biochem Biophys Res Commun. 2021 May 10;560:99-104. doi: 10.1016/j.bbrc.2021.04.124. Online ahead of print.ABSTRACTAggregates of mutant huntingtin (mHTT) containing an expanded polyglutamine (polyQ) tract are hallmarks of Huntington's Disease (HD). Studies have shown that mHTT can spread between cells, leading to the propagation of misfolded protein pathology. However, the structure of ... read more

J Gen Physiol. 2021 Jun 7;153(6):e202012843. doi: 10.1085/jgp.202012843. Epub 2021 May 12.NO ABSTRACTPMID:33978682 | DOI:10.1085/jgp.202012843 ... read more

Ageing Res Rev. 2021 May 9:101358. doi: 10.1016/j.arr.2021.101358. Online ahead of print.ABSTRACTHuntington disease (HD) is an autosomal dominant neurodegenerative disease that is caused by expansion of cytosine/adenosine/guanine repeats in the huntingtin (HTT) gene, which leads to a toxic, aggregation-prone, mutant HTT-polyQ protein. Beyond the well-established mechanisms of HD progression in ... read more

Hypersomnolence disorder (HD) is characterized by excessive sleep, which is a common sequela following stroke, infections or tumorigenesis. HD was traditionally thought to be associated with lesions of wake-promoting nuclei. However, lesion of a single, even two or more wake-promoting nucleuses simultaneously did not exert serious HD. The specific nucleus ... read more

Brain. 2021 May 11:awab186. doi: 10.1093/brain/awab186. Online ahead of print.ABSTRACTBrain cholesterol is produced mainly by astrocytes and is important for neuronal function. Its biosynthesis is severely reduced in mouse models of Huntington's disease. One possible mechanism is a diminished nuclear translocation of the transcription factor sterol regulatory element binding protein ... read more

Neurol Sci. 2021 May 11. doi: 10.1007/s10072-021-05289-w. Online ahead of print.ABSTRACTBACKGROUND: Cell nuclear architecture has been explored in cancer and laminopathies but not in neurodegenerative disorders. Huntington's disease (HD) is a neurodegenerative disorder that leads to neuronal death. Chromosome-wide changes in gene expression have been reported in HD, not only ... read more

Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2021 May 10;38(5):446-449. doi: 10.3760/cma.j.cn511374-20200323-00192.ABSTRACTOBJECTIVE: To explore the genetic basis for two Chinese pedigrees affected with Huntington disease and provide prenatal diagnosis for them.METHODS: Peripheral venous blood samples were collected from the probands. PCR and capillary gel electrophoresis were used to determine ... read more

Banana bunchy top virus (BBTV) is an important disease of banana in the Philippines and in other banana-producing countries. This study was conducted to investigate the genetic structure and diversity of Philippine BBTV isolates which remain unexplored in the country. BBTV-infected plant tissues were sampled from banana-growing provinces (i.e., Cagayan, ... read more

Arch Psychiatr Nurs. 2021 Jun;35(3):284-289. doi: 10.1016/j.apnu.2021.03.006. Epub 2021 Mar 26.ABSTRACTNeuropsychiatric manifestations of Huntington's disease (HD) can present years before motor symptoms. Nurses with specialized training provide superior care for HD patients, but HD exposure in nursing education is limited. Here we aimed to describe the historical neuropsychiatric burden in ... read more

J Vis Exp. 2021 Apr 25;(170). doi: 10.3791/62576.ABSTRACTProtein aggregation is a hallmark of neurodegenerative disorders such as amyotrophic lateral sclerosis (ALS), Alzheimer's disease (AD), Parkinson's disease (PD), Huntington's disease (HD), and so on. To detect and analyze soluble or diffuse protein oligomers or aggregates, fluorescence correlation spectroscopy (FCS), which can ... read more