(Q48454804)

11 February 2018

title

Altered gene expression in cells from patients with lysosomal storage disorders suggests impairment of the ubiquitin pathway. (English)

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11 February 2018

author

Robert Sladek

11

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11 February 2018

Sylvain Chemtob

8

object named as

S Chemtob

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11 February 2018

author name string

P Bifsha

1

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11 February 2018

K Landry

2

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11 February 2018

L Ashmarina

3

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11 February 2018

S Durand

4

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11 February 2018

V Seyrantepe

5

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11 February 2018

S Trudel

6

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11 February 2018

C Quiniou

7

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11 February 2018

Y Xu

9

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11 February 2018

R A Gravel

10

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11 February 2018

A V Pshezhetsky

12

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11 February 2018

publication date

4 August 2006

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11 February 2018

Cell Death & Differentiation

published in

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11 February 2018

volume

14

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11 February 2018

issue

3

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11 February 2018

page(s)

511-523

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11 February 2018

https://scigraph.springernature.com/pub.10.1038/sj.cdd.4402013

exact match

0 references

cites work

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Apoptotic cell death in mouse models of GM2 gangliosidosis and observations on human Tay-Sachs and Sandhoff diseases

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Identification of a molecular target of psychosine and its role in globoid cell formation

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Apoptotic positive cells in Krabbe brain and induction of apoptosis in rat C6 glial cells by psychosine

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Role of palmitoyl-protein thioesterase in cell death: implications for infantile neuronal ceroid lipofuscinosis

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Lysosomal cystine storage augments apoptosis in cultured human fibroblasts and renal tubular epithelial cells

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Lysosomal storage diseases: is impaired apoptosis a pathogenic mechanism?

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Microglial activation precedes acute neurodegeneration in Sandhoff disease and is suppressed by bone marrow transplantation

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Ubiquitin-protein conjugates accumulate in the lysosomal system of fibroblasts treated with cysteine proteinase inhibitors

21 January 2018

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Ubiquitinated protein conjugates are specifically enriched in the lysosomal system of fibroblasts

21 January 2018

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Ubiquitin carboxy-terminal hydrolase L1 binds to and stabilizes monoubiquitin in neuron

21 January 2018

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Activation of the cell death program by inhibition of proteasome function

21 January 2018

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21 January 2018

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Impairment of the ubiquitin-proteasome system by protein aggregation

21 January 2018

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The neuron-specific protein PGP 9.5 is a ubiquitin carboxyl-terminal hydrolase

21 January 2018

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Intragenic deletion in the gene encoding ubiquitin carboxy-terminal hydrolase in gad mice

21 January 2018

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Loss of Uch-L1 and Uch-L3 leads to neurodegeneration, posterior paralysis and dysphagia

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Identification of genes that modify ataxin-1-induced neurodegeneration.

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The UCH-L1 gene encodes two opposing enzymatic activities that affect alpha-synuclein degradation and Parkinson's disease susceptibility

21 January 2018

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UCH-L1 aggresome formation in response to proteasome impairment indicates a role in inclusion formation in Parkinson's disease

21 January 2018

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Regulation of apoptosis: the ubiquitous way

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Role of ubiquitin carboxy terminal hydrolase-L1 in neural cell apoptosis induced by ischemic retinal injury in vivo

21 January 2018

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Association of N-acetylgalactosamine-6-sulfate sulfatase with the multienzyme lysosomal complex of beta-galactosidase, cathepsin A, and neuraminidase. Possible implication for intralysosomal catabolism of keratan sulfate

21 January 2018

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Assessment of proteasome activity in cell lysates and tissue homogenates using peptide substrates

21 January 2018

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Niemann–Pick Disease versus acid sphingomyelinase deficiency

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7 January 2021

Pathological study of mice with total deficiency of sphingolipid activator proteins (SAP knockout mice).

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Evaluation of GM1 ganglioside-mediated apoptosis in feline thymocytes

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Severe type II Gaucher disease with ichthyosis, arthrogryposis and neuronal apoptosis: molecular and pathological analyses

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7 January 2021

Articular chondrocytes from animals with a dermatan sulfate storage disease undergo a high rate of apoptosis and release nitric oxide and inflammatory cytokines: a possible mechanism underlying degenerative joint disease in the mucopolysaccharidoses

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7 January 2021

Systemic inflammation in glucocerebrosidase-deficient mice with minimal glucosylceramide storage

1 reference

https://api.crossref.org/works/10.1038%2FSJ.CDD.4402013

7 January 2021

Oxidative modifications and down-regulation of ubiquitin carboxyl-terminal hydrolase L1 associated with idiopathic Parkinson's and Alzheimer's diseases

1 reference

https://api.crossref.org/works/10.1038%2FSJ.CDD.4402013

7 January 2021

Protein gene product 9.5 is expressed by fibroblasts in human cutaneous wounds

1 reference

https://api.crossref.org/works/10.1038%2FSJ.CDD.4402013

7 January 2021

Proteomic analysis of brain proteins in the gracile axonal dystrophy (gad) mouse, a syndrome that emanates from dysfunctional ubiquitin carboxyl-terminal hydrolase L-1, reveals oxidation of key proteins.

1 reference

https://api.crossref.org/works/10.1038%2FSJ.CDD.4402013

7 January 2021

Microarray expression analysis of gad mice implicates involvement of Parkinson's disease associated UCH-L1 in multiple metabolic pathways

1 reference

https://api.crossref.org/works/10.1038%2FSJ.CDD.4402013

7 January 2021

Identifiers

DOI

10.1038/SJ.CDD.4402013

1 reference

11 February 2018

release_dj4eoqm5b5gateffvnsfc6et3i

Fatcat ID

1 reference

Fatcat

https://api.fatcat.wiki/v0/release/dj4eoqm5b5gateffvnsfc6et3i

24 November 2022

mapped directly with Wikidata item

PubMed ID

16888648

1 reference

11 February 2018