UBQLN2/ubiquilin 2 mutation and pathology in familial amyotrophic lateral sclerosis.
about
The ubiquilin gene family: evolutionary patterns and functional insightsUbiquilin-2 (UBQLN2) binds with high affinity to the C-terminal region of TDP-43 and modulates TDP-43 levels in H4 cells: characterization of inhibition by nucleic acids and 4-aminoquinolinesUltrastructural studies of ALS mitochondria connect altered function and permeability with defects of mitophagy and mitochondriogenesisConverging mechanisms in ALS and FTD: disrupted RNA and protein homeostasisFrontotemporal dementia: a bridge between dementia and neuromuscular diseaseUbiquilin-2 drives NF-κB activity and cytosolic TDP-43 aggregation in neuronal cells.Protein Quality Control and the Amyotrophic Lateral Sclerosis/Frontotemporal Dementia ContinuumUBQLN2 mutation causing heterogeneous X-linked dominant neurodegeneration.Application of next-generation sequencing technologies in Neurology.Signature changes in ubiquilin expression in the R6/2 mouse model of Huntington's disease.Pathogenic Ubqln2 gains toxic properties to induce neuron death.Emerging evidence of coding mutations in the ubiquitin-proteasome system associated with cerebellar ataxiasCCNF mutations in amyotrophic lateral sclerosis and frontotemporal dementia.UBQLN2 Mediates Autophagy-Independent Protein Aggregate Clearance by the Proteasome.Clinical neurogenetics: amyotrophic lateral sclerosis.Next-generation sequencing in understanding complex neurological diseaseMotor neuron disease, TDP-43 pathology, and memory deficits in mice expressing ALS-FTD-linked UBQLN2 mutations.Protein aggregation in amyotrophic lateral sclerosis.Stress granules at the intersection of autophagy and ALS.TDP-43/FUS in motor neuron disease: Complexity and challenges.Autophagy Dysregulation in ALS: When Protein Aggregates Get Out of Hand.Impaired Mitophagy Plays a Role in Denervation of Neuromuscular Junctions in ALS Mice.Increased Ubqln2 expression causes neuron death in transgenic ratsThe Genetics of Monogenic Frontotemporal Dementia.Evaluation of Skin Fibroblasts from Amyotrophic Lateral Sclerosis Patients for the Rapid Study of Pathological Features.Causative Genes in Amyotrophic Lateral Sclerosis and Protein Degradation Pathways: a Link to Neurodegeneration.Complement components are upregulated and correlate with disease progression in the TDP-43Q331K mouse model of amyotrophic lateral sclerosis.Mutant UBQLN2 promotes toxicity by modulating intrinsic self-assembly
P2860
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P2860
UBQLN2/ubiquilin 2 mutation and pathology in familial amyotrophic lateral sclerosis.
description
2012 nî lūn-bûn
@nan
2012年の論文
@ja
2012年学术文章
@wuu
2012年学术文章
@zh-cn
2012年学术文章
@zh-hans
2012年学术文章
@zh-my
2012年学术文章
@zh-sg
2012年學術文章
@yue
2012年學術文章
@zh
2012年學術文章
@zh-hant
name
UBQLN2/ubiquilin 2 mutation and pathology in familial amyotrophic lateral sclerosis.
@en
UBQLN2/ubiquilin 2 mutation and pathology in familial amyotrophic lateral sclerosis.
@nl
type
label
UBQLN2/ubiquilin 2 mutation and pathology in familial amyotrophic lateral sclerosis.
@en
UBQLN2/ubiquilin 2 mutation and pathology in familial amyotrophic lateral sclerosis.
@nl
prefLabel
UBQLN2/ubiquilin 2 mutation and pathology in familial amyotrophic lateral sclerosis.
@en
UBQLN2/ubiquilin 2 mutation and pathology in familial amyotrophic lateral sclerosis.
@nl
P2093
P50
P1476
UBQLN2/ubiquilin 2 mutation and pathology in familial amyotrophic lateral sclerosis.
@en
P2093
Garth A Nicholson
Ian P Blair
Ruvini Fernando
Sadaf T Warraich
P304
2527.e3-10
P356
10.1016/J.NEUROBIOLAGING.2012.05.008
P577
2012-06-19T00:00:00Z