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Mutant huntingtin is secreted via a late endosomal/lysosomal unconventional secretory pathway. J Neurosci. 2017 Aug 16;: Authors: Trajkovic K, Jeong H, Krainc D Abstract Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by the expansion of a CAG triplet in the gene encoding for huntingtin. The resulting mutant ... read more

Comparison of the Toxic Effects of Quinolinic Acid and 3-Nitropropionic Acid in C. elegans: Involvement of the SKN-1 Pathway. Neurotox Res. 2017 Aug 18;: Authors: Kotlar I, Colonnello A, Aguilera-González MF, Avila DS, de Lima ME, García-Contreras R, Ortíz-Plata A, Soares FAA, Aschner M, Santamaría A Abstract The tryptophan metabolite, ... read more

Related ArticlesUsing Saccadometry with Deep Brain Stimulation to Study Normal and Pathological Brain Function. J Vis Exp. 2016 Jul 14;(113): Authors: Antoniades CA, FitzGerald JJ Abstract The oculomotor system involves a large number of brain areas including parts of the basal ganglia, and various neurodegenerative diseases including Parkinson's and ... read more

Related ArticlesPathogenic Huntington Alters BMP Signaling and Synaptic Growth through Local Disruptions of Endosomal Compartments. J Neurosci. 2017 Mar 22;37(12):3425-3439 Authors: Akbergenova Y, Littleton JT Abstract Huntington's disease (HD) is a neurodegenerative disorder caused by expansion of a polyglutamine (polyQ) stretch within the Huntingtin (Htt) protein. Pathogenic Htt disrupts ... read more

Related ArticlesReduced Expression of Foxp1 as a Contributing Factor in Huntington's Disease. J Neurosci. 2017 Jul 05;37(27):6575-6587 Authors: Louis Sam Titus ASC, Yusuff T, Cassar M, Thomas E, Kretzschmar D, D'Mello SR Abstract Huntington's disease (HD) is an inherited neurodegenerative disease caused by a polyglutamine expansion in the huntington ... read more

Are Astrocytes the Predominant Cell Type for Activation of Nrf2 in Aging and Neurodegeneration? Antioxidants (Basel). 2017 Aug 18;6(3): Authors: Liddell JR Abstract Nuclear factor erythroid 2-related factor 2 (Nrf2) is a transcription factor that regulates hundreds of antioxidant genes, and is activated in response to oxidative stress. Given that ... read more

Ribosomal transcription is regulated by PGC-1alpha and disturbed in Huntington's disease. Sci Rep. 2017 Aug 17;7(1):8513 Authors: Jesse S, Bayer H, Alupei MC, Zügel M, Mulaw M, Tuorto F, Malmsheimer S, Singh K, Steinacker J, Schumann U, Ludolph AC, Scharffetter-Kochanek K, Witting A, Weydt P, Iben S Abstract PGC-1α is ... read more

Related ArticlesMitochondrial targeting of XJB-5-131 attenuates or improves pathophysiology in HdhQ150 animals with well-developed disease phenotypes. Hum Mol Genet. 2016 May 01;25(9):1792-802 Authors: Polyzos A, Holt A, Brown C, Cosme C, Wipf P, Gomez-Marin A, Castro MR, Ayala-Peña S, McMurray CT Abstract Oxidative damage to mitochondria (MT) is ... read more

Related ArticlesMitochondria-targeted molecules MitoQ and SS31 reduce mutant huntingtin-induced mitochondrial toxicity and synaptic damage in Huntington's disease. Hum Mol Genet. 2016 May 01;25(9):1739-53 Authors: Yin X, Manczak M, Reddy PH Abstract The objective of this study was to determine the protective effects of the mitochondria-targeted molecules MitoQ and ... read more

Related ArticlesαB-Crystallin overexpression in astrocytes modulates the phenotype of the BACHD mouse model of Huntington's disease. Hum Mol Genet. 2016 May 01;25(9):1677-89 Authors: Oliveira AO, Osmand A, Outeiro TF, Muchowski PJ, Finkbeiner S Abstract Huntington's disease (HD) is caused by an expanded polyglutamine (polyQ) tract in the huntingtin ... read more

Related ArticlesMotor deficits associated with Huntington's disease occur in the absence of striatal degeneration in BACHD transgenic mice. Hum Mol Genet. 2016 May 01;25(9):1780-91 Authors: Mantovani S, Gordon R, Li R, Christie DC, Kumar V, Woodruff TM Abstract Huntington's disease (HD) is an incurable neurodegenerative condition characterized by progressive ... read more

Related ArticlesHuntington's Disease-Update on Treatments. Curr Neurol Neurosci Rep. 2017 Apr;17(4):33 Authors: Wyant KJ, Ridder AJ, Dayalu P Abstract Huntington's disease (HD) is an autosomal dominantly inherited neurodegenerative disease characterized by progressive motor, behavioral, and cognitive decline, ending in death. Despite the discovery of the underlying genetic mutation more ... read more

Related ArticlesA new Caenorhabditis elegans model of human huntingtin 513 aggregation and toxicity in body wall muscles. PLoS One. 2017;12(3):e0173644 Authors: Lee AL, Ung HM, Sands LP, Kikis EA Abstract Expanded polyglutamine repeats in different proteins are the known determinants of at least nine progressive neurodegenerative disorders whose ... read more

Huntington's disease: a clinical review. Eur J Neurol. 2017 Aug 17;: Authors: McColgan P, Tabrizi SJ Abstract Huntington's disease (HD) is a fully penetrant neurodegenerative disease caused by a dominantly inherited CAG trinucleotide repeat expansion in the huntingtin gene on chromosome 4. In Western populations HD has a prevalence of ... read more

Related ArticlesAn Exploration of Latent Structure in Observational Huntington's Disease Studies. AMIA Jt Summits Transl Sci Proc. 2017;2017:92-102 Authors: Ghosh S, Sun Z, Li Y, Cheng Y, Mohan A, Sampaio C, Hu J Abstract Huntington's disease (HD) is a monogenic neurodegenerative disorder characterized by the progressive decay of motor and ... read more

Related Articles2,4 DNP improves motor function, preserves medium spiny neuronal identity, and reduces oxidative stress in a mouse model of Huntington's disease. Exp Neurol. 2017 Jul;293:83-90 Authors: Wu B, Jiang M, Peng Q, Li G, Hou Z, Milne GL, Mori S, Alonso R, Geisler JG, Duan W Abstract Huntington's ... read more

Nicotinamide alone accelerates the conversion of mouse embryonic stem cells into mature neuronal populations. PLoS One. 2017;12(8):e0183358 Authors: Griffin SM, Pickard MR, Orme RP, Hawkins CP, Williams AC, Fricker RA Abstract INTRODUCTION: Vitamin B3 has been shown to play an important role during embryogenesis. Specifically, there is growing evidence that ... read more

Related ArticlesCryotolerance of Sperm from Transgenic Rhesus Macaques (Macaca mulatta). J Am Assoc Lab Anim Sci. 2016;55(5):520-4 Authors: Moran SP, Chi T, Prucha MS, Agca Y, Chan AW Abstract Cryopreservation is an important tool routinely used in preserving sperm for assisted reproductive technologies and for genetic preservation of ... read more

Related ArticlesAdverse mental health outcomes in breast cancer survivors compared to women who did not have cancer: systematic review protocol. Syst Rev. 2017 Aug 14;6(1):162 Authors: Carreira H, Williams R, Müller M, Harewood R, Bhaskaran K Abstract BACKGROUND: Recent increasing trends in breast cancer incidence and survival have resulted in ... read more

Related ArticlesThe expanded CAG repeat in the huntingtin gene as target for therapeutic RNA modulation throughout the HD mouse brain. PLoS One. 2017;12(2):e0171127 Authors: Datson NA, González-Barriga A, Kourkouta E, Weij R, van de Giessen J, Mulders S, Kontkanen O, Heikkinen T, Lehtimäki K, van Deutekom JC Abstract ... read more

Related ArticlesSubjects at Risk for Genetic Late-Onset Neurological Diseases: Objective Knowledge. Public Health Genomics. 2017 Aug 17;: Authors: Leite Â, Leite F, Dinis MAP Abstract BACKGROUND/AIMS: This study addresses the objective knowledge about the disease of subjects at risk for 3 genetic late-onset neurological diseases (LOND): familial amyloid polyneuropathy (FAP) ... read more

Related ArticlesDiffusion tensor imaging of brain white matter in Huntington gene mutation individuals. Arq Neuropsiquiatr. 2017 Aug;75(8):503-508 Authors: Saba RA, Yared JH, Doring TM, Phys M, Borges V, Ferraz HB Abstract Objective: To evaluate the role of the involvement of white matter tracts in huntingtin gene mutation patients as a ... read more

Related ArticlesFunctional Indicators of Glutamate Transport in Single Striatal Astrocytes and the Influence of Kir4.1 in Normal and Huntington Mice. J Neurosci. 2016 May 04;36(18):4959-75 Authors: Dvorzhak A, Vagner T, Kirmse K, Grantyn R Abstract UNLABELLED: This study evaluates single-cell indicators of glutamate transport in sulforhodamine 101-positive astrocytes of ... read more

Related ArticlesChronic subdural hematomas in Finnish patients with Huntington's disease. Acta Neurochir (Wien). 2016 Aug;158(8):1487-90 Authors: Sipilä JO, Posti JP, Majamaa K Abstract BACKGROUND: Many features of Huntington's disease (HD) make these patients susceptible to subdural hematomas (SDH), but there are no previous reports on the epidemiology of SDH ... read more

Related ArticlesCompromised Dopaminergic Encoding of Reward Accompanying Suppressed Willingness to Overcome High Effort Costs Is a Prominent Prodromal Characteristic of the Q175 Mouse Model of Huntington's Disease. J Neurosci. 2016 May 04;36(18):4993-5002 Authors: Covey DP, Dantrassy HM, Zlebnik NE, Gildish I, Cheer JF Abstract UNLABELLED: Huntington's disease (HD) is ... read more

Related ArticlesCorrelations of Behavioral Deficits with Brain Pathology Assessed through Longitudinal MRI and Histopathology in the HdhQ150/Q150 Mouse Model of Huntington's Disease. PLoS One. 2017;12(1):e0168556 Authors: Rattray I, Smith EJ, Crum WR, Walker TA, Gale R, Bates GP, Modo M Abstract A variety of mouse models have been ... read more

Related ArticlesThe BACHD Rat Model of Huntington Disease Shows Signs of Fronto-Striatal Dysfunction in Two Operant Conditioning Tests of Short-Term Memory. PLoS One. 2017;12(1):e0169051 Authors: Clemensson EK, Clemensson LE, Riess O, Nguyen HP Abstract The BACHD rat is a recently developed transgenic animal model of Huntington disease, a ... read more

Related ArticlesStatistical Approaches to Longitudinal Data Analysis in Neurodegenerative Diseases: Huntington's Disease as a Model. Curr Neurol Neurosci Rep. 2017 Feb;17(2):14 Authors: Garcia TP, Marder K Abstract Understanding the overall progression of neurodegenerative diseases is critical to the timing of therapeutic interventions and design of effective clinical trials. Disease ... read more

Related ArticlesTherapeutic targeting of the pathological triad of extrasynaptic NMDA receptor signaling in neurodegenerations. J Exp Med. 2017 Mar 06;214(3):569-578 Authors: Bading H Abstract Activation of extrasynaptic N-methyl-d-aspartate (NMDA) receptors causes neurodegeneration and cell death. The disease mechanism involves a pathological triad consisting of mitochondrial dysfunction, loss of integrity ... read more

Related ArticlesEpidemiological Study of Huntington's Disease in the Province of Ferrara, Italy. Neuroepidemiology. 2017 Aug 12;49(1-2):18-23 Authors: Carrassi E, Pugliatti M, Govoni V, Sensi M, Casetta I, Granieri E Abstract BACKGROUND: Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by the abnormal expansion of CAG triplet repeat. We ... read more

Related ArticlesHuntington Disease: Linking Pathogenesis to the Development of Experimental Therapeutics. Curr Neurol Neurosci Rep. 2017 Feb;17(2):18 Authors: Mestre TA, Sampaio C Abstract Huntington disease (HD) is an autosomal dominant neurodegenerative condition caused by a CAG trinucleotide expansion in the huntingtin gene. At present, the HD field is experiencing ... read more

Related ArticlesDiversity of astroglial responses across human neurodegenerative disorders and brain aging. Brain Pathol. 2017 Sep;27(5):645-674 Authors: Ferrer I Abstract Astrogliopathy refers to alterations of astrocytes occurring in diseases of the nervous system, and it implies the involvement of astrocytes as key elements in the pathogenesis and pathology of diseases ... read more

Related ArticlesElimination of Toxic Microsatellite Repeat Expansion RNA by RNA-Targeting Cas9. Cell. 2017 Aug 08;: Authors: Batra R, Nelles DA, Pirie E, Blue SM, Marina RJ, Wang H, Chaim IA, Thomas JD, Zhang N, Nguyen V, Aigner S, Markmiller S, Xia G, Corbett KD, Swanson MS, Yeo GW Abstract Microsatellite ... read more

Nonmotor Symptoms in Huntington Disease. Int Rev Neurobiol. 2017;134:1397-1408 Authors: Cardoso F Abstract Huntington disease (HD) is characterized by the triad of motor abnormalities, cognitive dysfunction, and behavioral changes. The aim of this chapter is to describe the frequency, clinical features, and management of behavioral and cognitive dysfunction in HD. ... read more

Related ArticlesNuclear factor (erythroid-derived 2)-like 2 (NRF2) drug discovery: Biochemical toolbox to develop NRF2 activators by reversible binding of Kelch-like ECH-associated protein 1 (KEAP1). Arch Biochem Biophys. 2017 Aug 08;: Authors: Bresciani A, Missineo A, Gallo M, Cerretani M, Fezzardi P, Tomei L, Cicero DO, Altamura S, Santoprete A, Ingenito ... read more

Related ArticlesInsulin-Like Growth Factor-1 but Not Insulin Predicts Cognitive Decline in Huntington's Disease. PLoS One. 2016;11(9):e0162890 Authors: Salem L, Saleh N, Désaméricq G, Youssov K, Dolbeau G, Cleret L, Bourhis ML, Azulay JP, Krystkowiak P, Verny C, Morin F, Moutereau S, French Huntington Study Group, Bachoud-Lévi AC, Maison ... read more

Related ArticlesImaging and spectroscopic approaches to probe brain energy metabolism dysregulation in neurodegenerative diseases. J Cereb Blood Flow Metab. 2017 Jun;37(6):1927-1943 Authors: Bonvento G, Valette J, Flament J, Mochel F, Brouillet E Abstract Changes in energy metabolism are generally considered to play an important role in neurodegenerative diseases such as ... read more

Related ArticlesThe FOXP2-Driven Network in Developmental Disorders and Neurodegeneration. Front Cell Neurosci. 2017;11:212 Authors: Oswald F, Klöble P, Ruland A, Rosenkranz D, Hinz B, Butter F, Ramljak S, Zechner U, Herlyn H Abstract The transcription repressor FOXP2 is a crucial player in nervous system evolution and development of humans and ... read more

Related ArticlesMitophagy in neurodegenerative diseases. Neurochem Int. 2017 Aug 07;: Authors: Rodolfo C, Campello S, Cecconi F Abstract Neurodegenerative diseases, such as Parkinson's disease (PD), Alzheimer's disease (AD), Huntington's disease (HD), and Amyotrophic Lateral Sclerosis (ALS), are a complex "family" of pathologies, characterised by the progressive loss of neurons and/or ... read more

Mitochondrial Dynamics and Proteins Related to Neurodegenerative Diseases. Curr Protein Pept Sci. 2017 Aug 10;: Authors: Alexiou A, Nizami B, Khan FI, Soursou G, Vairaktarakis C, Chatzichronis S, Tsiamis V, Manztavinos V, Yarla NS, Ashraf GM Abstract Disruptions in the regulation of mitochondrial dynamics and the occurrence of proteins misfolding ... read more

Evolving Motivations: Patients' and Caregivers' Perceptions About Seeking Myotonic Dystrophy (DM1) and Huntington's Disease Care. Qual Health Res. 2017 Sep;27(11):1727-1737 Authors: LaDonna KA, Watling CJ, Ray SL, Piechowicz C, Venance SL Abstract Patient-centered care provision is challenging under ideal circumstances; myotonic dystrophy (DM1) and Huntington's disease (HD) are examples of ... read more

Related ArticlesStriatal Network Models of Huntington's Disease Dysfunction Phenotypes. Front Comput Neurosci. 2017;11:70 Authors: Zheng P, Kozloski J Abstract We present a network model of striatum, which generates "winnerless" dynamics typical for a network of sparse, unidirectionally connected inhibitory units. We observe that these dynamics, while interesting and a good ... read more

Related ArticlesYeast buddies helping to unravel the complexity of neurodegenerative disorders. Mech Ageing Dev. 2017 Jan;161(Pt B):288-305 Authors: Fruhmann G, Seynnaeve D, Zheng J, Ven K, Molenberghs S, Wilms T, Liu B, Winderickx J, Franssens V Abstract Neurodegenerative disorders have a profound effect on the quality of life of ... read more

Related ArticlesHuntington disease. JAAPA. 2017 Mar;30(3):46-47 Authors: Nold CS PMID: 28221321 [PubMed - indexed for MEDLINE] ... read more

Cross-seeding of prions by aggregated α-synuclein leads to transmissible spongiform encephalopathy. PLoS Pathog. 2017 Aug 10;13(8):e1006563 Authors: Katorcha E, Makarava N, Lee YJ, Lindberg I, Monteiro MJ, Kovacs GG, Baskakov IV Abstract Aggregation of misfolded proteins or peptides is a common feature of neurodegenerative diseases including Alzheimer's, Parkinson's, Huntington's, prion ... read more

Related ArticlesMdivi-1 ameliorates early brain injury after subarachnoid hemorrhage via the suppression of inflammation-related blood-brain barrier disruption and endoplasmic reticulum stress-based apoptosis. Free Radic Biol Med. 2017 Aug 05;: Authors: Fan LF, He PY, Peng YC, Du QH, Ma YJ, Jin JX, Xu HZ, Li JR, Wang ZJ, Cao SL, ... read more

Related ArticlesTics as an initial manifestation of juvenile Huntington's disease: case report and literature review. BMC Neurol. 2017 Aug 08;17(1):152 Authors: Cui SS, Ren RJ, Wang Y, Wang G, Chen SD Abstract BACKGROUND: Huntington's disease (HD) is an autosomal dominant disorder, typically characterized by chorea due to a trinucleotide repeat ... read more

Induced neural stem cells as a means of treatment in Huntington's disease. Expert Opin Biol Ther. 2017 Aug 09;:1-11 Authors: Choi KA, Hong S Abstract INTRODUCTION: Huntington's disease (HD) is an inherited neurodegenerative disease characterized by chorea, dementia, and depression caused by progressive nerve cell degeneration, which is triggered by ... read more

Related ArticlesMelatonin as a mitochondrial protector in neurodegenerative diseases. Cell Mol Life Sci. 2017 Aug 08;: Authors: Wongprayoon P, Govitrapong P Abstract Mitochondria are crucial organelles as their role in cellular energy production of eukaryotes. Because the brain cells demand high energy for maintaining their normal activities, disturbances in mitochondrial ... read more

Related ArticlesGenetics of disease severity in multiple sclerosis, Alzheimer's disease, and Huntington's disease: rejuvenating genome-wide association studies. J Neurol. 2017 Aug 07;: Authors: Hrastelj J, Robertson N PMID: 28785799 [PubMed - as supplied by publisher] ... read more