A molecular investigation of true dominance in Huntington's disease. - Wikidata - awgldk - TriplyDB

A molecular investigation of true dominance in Huntington's disease.

A molecular investigation of true dominance in Huntington's disease.

http://www.wikidata.org/entity/Q35430697

about

Laforin, the most common protein mutated in Lafora disease, regulates autophagy PI(5)P regulates autophagosome biogenesis Autophagic substrate clearance requires activity of the syntaxin-5 SNARE complex Genetics and neuropathology of Huntington's disease A universal mechanism ties genotype to phenotype in trinucleotide diseases A block of autophagy in lysosomal storage disorders C/EBPβ regulates sensitivity to bortezomib in prostate cancer cells by inducing REDD1 and autophagosome-lysosome fusion Cellular localization and development of neuronal intranuclear inclusions in striatal and cortical neurons in R6/2 transgenic mice Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro Intrinsically disordered proteins as molecular shields Deletion of the huntingtin polyglutamine stretch enhances neuronal autophagy and longevity in mice.Inactivation of Drosophila Huntingtin affects long-term adult functioning and the pathogenesis of a Huntington's disease model.Effects of overexpression of huntingtin proteins on mitochondrial integrity.Mutation in VPS35 associated with Parkinson's disease impairs WASH complex association and inhibits autophagy PICALM modulates autophagy activity and tau accumulation.Calpain inhibition mediates autophagy-dependent protection against polyglutamine toxicity.Bromodomain Protein BRD4 Is a Transcriptional Repressor of Autophagy and Lysosomal Function.An aggregation sensing reporter identifies leflunomide and teriflunomide as polyglutamine aggregate inhibitors.Puromycin-sensitive aminopeptidase protects against aggregation-prone proteins via autophagy Dynein mutations impair autophagic clearance of aggregate-prone proteins.Small molecules enhance autophagy and reduce toxicity in Huntington's disease models.Conophylline protects cells in cellular models of neurodegenerative diseases by inducing mammalian target of rapamycin (mTOR)-independent autophagy.Bacterial and yeast chaperones reduce both aggregate formation and cell death in mammalian cell models of Huntington's disease.The Parkinson's disease protein LRRK2 impairs proteasome substrate clearance without affecting proteasome catalytic activity.CAG repeat lengths > or =335 attenuate the phenotype in the R6/2 Huntington's disease transgenic mouse.Polyglutamine aggregates alter protein folding homeostasis in Caenorhabditis elegans.Hydrophilic protein associated with desiccation tolerance exhibits broad protein stabilization function Lithium induces autophagy by inhibiting inositol monophosphatase siRNA screen identifies QPCT as a druggable target for Huntington's disease.The Hedgehog signalling pathway regulates autophagy.Rab5 modulates aggregation and toxicity of mutant huntingtin through macroautophagy in cell and fly models of Huntington disease.ALS-associated mutant FUS inhibits macroautophagy which is restored by overexpression of Rab1 Dominant versus recessive: molecular mechanisms in metabolic disease.Onjisaponin B derived from Radix Polygalae enhances autophagy and accelerates the degradation of mutant α-synuclein and huntingtin in PC-12 cells.Azithromycin blocks autophagy and may predispose cystic fibrosis patients to mycobacterial infection.Trehalose reduces aggregate formation and delays pathology in a transgenic mouse model of oculopharyngeal muscular dystrophy.Accumulation of autophagosomes confers cytotoxicity.Neferine attenuates the protein level and toxicity of mutant huntingtin in PC-12 cells via induction of autophagy.IGF-1 receptor antagonism inhibits autophagy.Measurement of autophagic activity in mammalian cells.

P2860

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P2860

A molecular investigation of true dominance in Huntington's disease.

http://www.wikidata.org/entity/Q35430697

description

1999 nî lūn-bûn

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1999 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած

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1999 թվականի հոտեմբերին հրատարակված գիտական հոդված

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1999年の論文

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1999年論文

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1999年論文

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1999年論文

@zh-hk

1999年論文

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1999年論文

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1999年论文

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name

A molecular investigation of true dominance in Huntington's disease.

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A molecular investigation of true dominance in Huntington's disease.

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type

label

A molecular investigation of true dominance in Huntington's disease.

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A molecular investigation of true dominance in Huntington's disease.

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prefLabel

A molecular investigation of true dominance in Huntington's disease.

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A molecular investigation of true dominance in Huntington's disease.

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A molecular investigation of true dominance in Huntington's disease.

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A Wyttenbach

http://www.w3.org/2001/XMLSchema#string

D C Rubinsztein

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J Rankin

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R A Furlong

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Y Narain

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P2860

Genotypes at the GluR6 kainate receptor locus are associated with variation in the age of onset of Huntington disease

Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation

Targeted disruption of the Huntington's disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes

Increased apoptosis and early embryonic lethality in mice nullizygous for the Huntington's disease gene homologue

Inactivation of the mouse Huntington's disease gene homolog Hdh

Huntingtin is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion

Glutamine repeats and neurodegenerative diseases: molecular aspects.

Intracellular inclusions, pathological markers in diseases caused by expanded polyglutamine tracts?

Unstable triplet repeat and phenotypic variability of spinocerebellar ataxia type 1.

Identification of the spinocerebellar ataxia type 2 gene using a direct identification of repeat expansion and cloning technique, DIRECT.

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10528852

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Huntington disease

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1734229

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