Recessive loss of function of the neuronal ubiquitin hydrolase UCHL1 leads to early-onset progressive neurodegeneration
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Biallelic mutations in SNX14 cause a syndromic form of cerebellar atrophy and lysosome-autophagosome dysfunctionUbiquitin-Synaptobrevin Fusion Protein Causes Degeneration of Presynaptic Motor Terminals in MiceUbiquitin C-terminal hydrolase L1 (UCH-L1): structure, distribution and roles in brain function and dysfunctionProteomics in Traditional Chinese Medicine with an Emphasis on Alzheimer's DiseaseCorticospinal Motor Neurons Are Susceptible to Increased ER Stress and Display Profound Degeneration in the Absence of UCHL1 FunctionNon-coding RNA in neural function, disease, and agingUCHL1 deficiency exacerbates human islet amyloid polypeptide toxicity in β-cells: evidence of interplay between the ubiquitin/proteasome system and autophagyUnraveling the genetic landscape of autosomal recessive Charcot-Marie-Tooth neuropathies using a homozygosity mapping approach.In vivo transduction of neurons with TAT-UCH-L1 protects brain against controlled cortical impact injury.An optimal ubiquitin-proteasome pathway in the nervous system: the role of deubiquitinating enzymes.Military personnel with chronic symptoms following blast traumatic brain injury have differential expression of neuronal recovery and epidermal growth factor receptor genes.Genes and genetic testing in hereditary ataxias.Parkin-mediated K63-polyubiquitination targets ubiquitin C-terminal hydrolase L1 for degradation by the autophagy-lysosome system.Phosphoproteomic profiling of selenate-treated Alzheimer's disease model cells.eGFP expression under UCHL1 promoter genetically labels corticospinal motor neurons and a subpopulation of degeneration-resistant spinal motor neurons in an ALS mouse model.Endogenous neurotoxic dopamine derivative covalently binds to Parkinson's disease-associated ubiquitin C-terminal hydrolase L1 and alters its structure and function.The point mutation UCH-L1 C152A protects primary neurons against cyclopentenone prostaglandin-induced cytotoxicity: implications for post-ischemic neuronal injury.Spinal Muscular Atrophy Patient iPSC-Derived Motor Neurons Have Reduced Expression of Proteins Important in Neuronal Development.Emerging evidence of coding mutations in the ubiquitin-proteasome system associated with cerebellar ataxiasUbiquitin C-terminal hydrolase L1 (UCH-L1) acts as a novel potentiator of cyclin-dependent kinases to enhance cell proliferation independently of its hydrolase activity.Absence of UCHL 1 function leads to selective motor neuropathy.Deubiquitylases from genes to organism.Genetic landscape remodelling in spinocerebellar ataxias: the influence of next-generation sequencing.Emerging bioinformatics approaches for analysis of NGS-derived coding and non-coding RNAs in neurodegenerative diseases.Identification and functional analysis of a potential key lncRNA involved in fat loss of cancer cachexia.Advances in understanding the role of disease-associated proteins in spinal muscular atrophy.The fine-tuning of proteolytic pathways in Alzheimer's disease.Proteomic Analysis After Status Epilepticus Identifies UCHL1 as Protective Against Hippocampal Injury.Life and death in the trash heap: The ubiquitin proteasome pathway and UCHL1 in brain aging, neurodegenerative disease and cerebral Ischemia.Overcoming the divide between ataxias and spastic paraplegias: Shared phenotypes, genes, and pathways.Modification of ubiquitin C-terminal hydrolase L1 by reactive lipid species: role in neural regeneration and diseases of aging.Expression analysis of the long non-coding RNA antisense to Uchl1 (AS Uchl1) during dopaminergic cells' differentiation in vitro and in neurochemical models of Parkinson's diseaseOverexpression of ubiquitin carboxyl-terminal hydrolase L1 (UCHL1) delays Alzheimer's progression in vivo.UCHL1 expression and localization on testicular development and spermatogenesis of Chinese giant salamanders.UCH-L1 promotes invasion of breast cancer cells through activating Akt signaling pathway.Association of ubiquitin carboxy-terminal hydrolase-L1 in cerebrospinal fluid with clinical severity in a cohort of patients with Guillain-Barré syndrome.Comparative efficacy of alpha-linolenic acid and gamma-linolenic acid to attenuate valproic acid-induced autism-like features.Rare Disease Mechanisms Identified by Genealogical Proteomics of Copper Homeostasis Mutant Pedigrees.UCH-L1 Inhibition Suppresses tau Aggresome Formation during Proteasomal Impairment.Role of the Ubiquitin C-Terminal Hydrolase L1-Modulated Ubiquitin Proteasome System in Auditory Cortex Senescence.
P2860
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P2860
Recessive loss of function of the neuronal ubiquitin hydrolase UCHL1 leads to early-onset progressive neurodegeneration
description
2013 nî lūn-bûn
@nan
2013 թուականի Յունուարին հրատարակուած գիտական յօդուած
@hyw
2013 թվականի հունվարին հրատարակված գիտական հոդված
@hy
2013年の論文
@ja
2013年論文
@yue
2013年論文
@zh-hant
2013年論文
@zh-hk
2013年論文
@zh-mo
2013年論文
@zh-tw
2013年论文
@wuu
name
Recessive loss of function of ...... progressive neurodegeneration
@ast
Recessive loss of function of ...... progressive neurodegeneration
@en
Recessive loss of function of ...... progressive neurodegeneration
@nl
type
label
Recessive loss of function of ...... progressive neurodegeneration
@ast
Recessive loss of function of ...... progressive neurodegeneration
@en
Recessive loss of function of ...... progressive neurodegeneration
@nl
prefLabel
Recessive loss of function of ...... progressive neurodegeneration
@ast
Recessive loss of function of ...... progressive neurodegeneration
@en
Recessive loss of function of ...... progressive neurodegeneration
@nl
P2093
P2860
P50
P356
P1476
Recessive loss of function of ...... progressive neurodegeneration
@en
P2093
Ahmet O Caglayan
Alp Dincer
Angeliki Louvi
Beyhan Tuysuz
Cigdem Ozkara
Jacob F Baranoski
Mehmet Bakircioglu
Michele H Johnson
Murat Gunel
Murat Karacorlu
P2860
P304
P356
10.1073/PNAS.1222732110
P407
P577
2013-01-28T00:00:00Z