Decreased glucocerebrosidase activity in Gaucher disease parallels quantitative enzyme loss due to abnormal interaction with TCP1 and c-Cbl.
about
Glucocerebrosidase is shaking up the synucleinopathiesInhibition of endoplasmic reticulum-associated degradation rescues native folding in loss of function protein misfolding diseasesImpairment of homeostasis in lysosomal storage disordersGaucher disease and malignancy: a model for cancer pathogenesis in an inborn error of metabolismβ-Catenin signaling initiates the activation of astrocytes and its dysregulation contributes to the pathogenesis of astrocytomas.Missense mutations in the NF2 gene result in the quantitative loss of merlin protein and minimally affect protein intrinsic function.Parkin-mediated ubiquitination of mutant glucocerebrosidase leads to competition with its substrates PARIS and ARTSMutant glucocerebrosidase in Gaucher disease recruits Hsp27 to the Hsp90 chaperone complex for proteasomal degradationProtective role of AMP-activated protein kinase-evoked autophagy on an in vitro model of ischemia/reperfusion-induced renal tubular cell injury.Pharmacological chaperone therapy for lysosomal storage diseases.Histone deacetylase inhibitors prevent the degradation and restore the activity of glucocerebrosidase in Gaucher diseaseLysosomal Enzyme Glucocerebrosidase Protects against Aβ1-42 Oligomer-Induced Neurotoxicity.Missense mutations in the human SDHB gene increase protein degradation without altering intrinsic enzymatic function.Histone deacetylase inhibitors increase glucocerebrosidase activity in Gaucher disease by modulation of molecular chaperonesAn innovative approach to the treatment of Gaucher disease and possibly other metabolic disorders of the brain.Glucocerebrosidase deficiency in substantia nigra of parkinson disease brains.Hypotaurine evokes a malignant phenotype in glioma through aberrant hypoxic signaling.Parkinson disease-linked GBA mutation effects reversed by molecular chaperones in human cell and fly models.Celastrol increases glucocerebrosidase activity in Gaucher disease by modulating molecular chaperones.A Guided Tour of the Structural Biology of Gaucher Disease: Acid-β-Glucosidase and Saposin C.Glucocerebrosidase inhibitors for the treatment of Gaucher disease.New Directions in Gaucher Disease.Bicyclic derivatives of L-idonojirimycin as pharmacological chaperones for neuronopathic forms of Gaucher disease.Association of CCT/TriC with other substrates during biosynthesis (unknown chaperone)Dermal fibroblasts from patients with Parkinson's disease have normal GCase activity and autophagy compared to patients with PD and GBA mutations.D409H GBA1 mutation accelerates the progression of pathology in A53T α-synuclein transgenic mouse model.
P2860
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P2860
Decreased glucocerebrosidase activity in Gaucher disease parallels quantitative enzyme loss due to abnormal interaction with TCP1 and c-Cbl.
description
2010 nî lūn-bûn
@nan
2010 թուականի Դեկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2010 թվականի դեկտեմբերին հրատարակված գիտական հոդված
@hy
2010年の論文
@ja
2010年論文
@yue
2010年論文
@zh-hant
2010年論文
@zh-hk
2010年論文
@zh-mo
2010年論文
@zh-tw
2010年论文
@wuu
name
Decreased glucocerebrosidase a ...... teraction with TCP1 and c-Cbl.
@ast
Decreased glucocerebrosidase a ...... teraction with TCP1 and c-Cbl.
@en
Decreased glucocerebrosidase a ...... teraction with TCP1 and c-Cbl.
@nl
type
label
Decreased glucocerebrosidase a ...... teraction with TCP1 and c-Cbl.
@ast
Decreased glucocerebrosidase a ...... teraction with TCP1 and c-Cbl.
@en
Decreased glucocerebrosidase a ...... teraction with TCP1 and c-Cbl.
@nl
altLabel
Decreased glucocerebrosidase a ...... nteraction with TCP1 and c-Cbl
@en
prefLabel
Decreased glucocerebrosidase a ...... teraction with TCP1 and c-Cbl.
@ast
Decreased glucocerebrosidase a ...... teraction with TCP1 and c-Cbl.
@en
Decreased glucocerebrosidase a ...... teraction with TCP1 and c-Cbl.
@nl
P2093
P2860
P50
P3181
P356
P1476
Decreased glucocerebrosidase a ...... nteraction with TCP1 and c-Cbl
@en
P2093
Chunzhang Yang
Eli Thompson
Jeffrey Chiang
Masako Chen
Rajiv R Iyer
Roscoe O Brady
Russell R Lonser
Zhengping Zhuang
P2860
P304
21665-21670
P3181
P356
10.1073/PNAS.1014376107
P407
P577
2010-11-22T00:00:00Z