Topology and endoplasmic reticulum retention signals of the lysosomal storage disease-related membrane protein CLN6.
about
Protein product of CLN6 gene responsible for variant late-onset infantile neuronal ceroid lipofuscinosis interacts with CRMP-2Kufs disease, the major adult form of neuronal ceroid lipofuscinosis, caused by mutations in CLN6A murine model of variant late infantile ceroid lipofuscinosis recapitulates behavioral and pathological phenotypes of human diseaseInteractions of the proteins of neuronal ceroid lipofuscinosis: clues to functionCytosolic N-terminal arginine-based signals together with a luminal signal target a type II membrane protein to the plant ER.Disruption of the autophagy-lysosome pathway is involved in neuropathology of the nclf mouse model of neuronal ceroid lipofuscinosisOpening Pandora's jar: a primer on the putative roles of CRMP2 in a panoply of neurodegenerative, sensory and motor neuron, and central disorders.Cln6 mutants associated with neuronal ceroid lipofuscinosis are degraded in a proteasome-dependent manner.Update of the mutation spectrum and clinical correlations of over 360 mutations in eight genes that underlie the neuronal ceroid lipofuscinoses.Pharmaceutical Chaperones and Proteostasis Regulators in the Therapy of Lysosomal Storage Disorders: Current Perspective and Future Promises.NCLs and ER: A stressful relationship.Identification of trafficking determinants for polytopic rhomboid proteases in Toxoplasma gondii.High expression of disease-related Cln6 in the cerebral cortex, purkinje cells, dentate gyrus, and hippocampal ca1 neurons.Running apraxia as a presenting symptom of neuronal ceroid lipofuscinosis 6.
P2860
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P2860
Topology and endoplasmic reticulum retention signals of the lysosomal storage disease-related membrane protein CLN6.
description
2007 nî lūn-bûn
@nan
2007 թուականի Յունուարին հրատարակուած գիտական յօդուած
@hyw
2007 թվականի հունվարին հրատարակված գիտական հոդված
@hy
2007年の論文
@ja
2007年論文
@yue
2007年論文
@zh-hant
2007年論文
@zh-hk
2007年論文
@zh-mo
2007年論文
@zh-tw
2007年论文
@wuu
name
Topology and endoplasmic retic ...... related membrane protein CLN6.
@ast
Topology and endoplasmic retic ...... related membrane protein CLN6.
@en
Topology and endoplasmic retic ...... related membrane protein CLN6.
@nl
type
label
Topology and endoplasmic retic ...... related membrane protein CLN6.
@ast
Topology and endoplasmic retic ...... related membrane protein CLN6.
@en
Topology and endoplasmic retic ...... related membrane protein CLN6.
@nl
prefLabel
Topology and endoplasmic retic ...... related membrane protein CLN6.
@ast
Topology and endoplasmic retic ...... related membrane protein CLN6.
@en
Topology and endoplasmic retic ...... related membrane protein CLN6.
@nl
P2093
P2860
P50
P1476
Topology and endoplasmic retic ...... related membrane protein CLN6.
@en
P2093
Arne Quitsch
Claudia Heine
Liina Lonka
Stephan Storch
Thomas Braulke
P2860
P356
10.1080/09687860600967317
P407
P577
2007-01-01T00:00:00Z