The peroxisome biogenesis disorder group 4 gene, PXAAA1, encodes a cytoplasmic ATPase required for stability of the PTS1 receptor
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Mutation in PEX16 is causal in the peroxisome-deficient Zellweger syndrome of complementation group D.Peroxisome synthesis in the absence of preexisting peroxisomesIdentification and characterization of the human orthologue of yeast Pex14pPEX12 interacts with PEX5 and PEX10 and acts downstream of receptor docking in peroxisomal matrix protein importPEX19 binds multiple peroxisomal membrane proteins, is predominantly cytoplasmic, and is required for peroxisome membrane synthesisAlternative splicing suggests extended function of PEX26 in peroxisome biogenesis.Pex13p is an SH3 protein of the peroxisome membrane and a docking factor for the predominantly cytoplasmic PTs1 receptorIdentification of PEX10, the gene defective in complementation group 7 of the peroxisome-biogenesis disordersPEX12, the pathogenic gene of group III Zellweger syndrome: cDNA cloning by functional complementation on a CHO cell mutant, patient analysis, and characterization of PEX12pTwo AAA family peroxins, PpPex1p and PpPex6p, interact with each other in an ATP-dependent manner and are associated with different subcellular membranous structures distinct from peroxisomes.Mutations in novel peroxin gene PEX26 that cause peroxisome-biogenesis disorders of complementation group 8 provide a genotype-phenotype correlationPeroxisome targeting signal type 1 (PTS1) receptor is involved in import of both PTS1 and PTS2: studies with PEX5-defective CHO cell mutants.PEX11alpha is required for peroxisome proliferation in response to 4-phenylbutyrate but is dispensable for peroxisome proliferator-activated receptor alpha-mediated peroxisome proliferationPhenotype-genotype relationships in complementation group 3 of the peroxisome-biogenesis disordersPEX13 is mutated in complementation group 13 of the peroxisome-biogenesis disorders.A PEX6-defective peroxisomal biogenesis disorder with severe phenotype in an infant, versus mild phenotype resembling Usher syndrome in the affected parentsHuman PEX1 cloned by functional complementation on a CHO cell mutant is responsible for peroxisome-deficient Zellweger syndrome of complementation group IA unified nomenclature for peroxisome biogenesis factorsDisruption of a PEX1-PEX6 interaction is the most common cause of the neurologic disorders Zellweger syndrome, neonatal adrenoleukodystrophy, and infantile Refsum diseaseMultiple PEX genes are required for proper subcellular distribution and stability of Pex5p, the PTS1 receptor: evidence that PTS1 protein import is mediated by a cycling receptorPeroxisomes in brain development and functionSaccharomyces cerevisiae pex3p and pex19p are required for proper localization and stability of peroxisomal membrane proteinsIdentification of novel mutations and sequence variation in the Zellweger syndrome spectrum of peroxisome biogenesis disordersPeroxisomal metabolic function is required for appressorium-mediated plant infection by Colletotrichum lagenariumMutational analysis of Cdc19p, a Schizosaccharomyces pombe MCM protein.Shuttling mechanism of peroxisome targeting signal type 1 receptor Pex5: ATP-independent import and ATP-dependent export.Different phenotypes in vivo are associated with ATPase motif mutations in Schizosaccharomyces pombe minichromosome maintenance proteinsDynamics of the peroxisomal import cycle of PpPex20p: ubiquitin-dependent localization and regulation.An Arabidopsis indole-3-butyric acid-response mutant defective in PEROXIN6, an apparent ATPase implicated in peroxisomal function.Inhibitors of COPI and COPII do not block PEX3-mediated peroxisome synthesis.Proteomic analysis reveals that the Rab GTPase RabE1c is involved in the degradation of the peroxisomal protein receptor PEX7 (peroxin 7).Fluorescent co-localization of PTS1 and PTS2 and its application in analysis of the gene function and the peroxisomal dynamic in Magnaporthe oryzae.Peroxisome biogenesis and human peroxisome-deficiency disorders.The peroxisome biogenesis factors pex4p, pex22p, pex1p, and pex6p act in the terminal steps of peroxisomal matrix protein import.Lymphoblastoid cell lines for diagnosis of peroxisome biogenesis disorders.Ubiquitination of the peroxisomal import receptor Pex5pTRIM37, a novel E3 ligase for PEX5-mediated peroxisomal matrix protein import.A New Yeast Peroxin, Pex36, a Functional Homolog of Mammalian PEX16, Functions in the ER-to-Peroxisome Traffic of Peroxisomal Membrane Proteins.Role of PEX5 ubiquitination in maintaining peroxisome dynamics and homeostasis.Chemically monoubiquitinated PEX5 binds to the components of the peroxisomal docking and export machinery
P2860
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P2860
The peroxisome biogenesis disorder group 4 gene, PXAAA1, encodes a cytoplasmic ATPase required for stability of the PTS1 receptor
description
1996 nî lūn-bûn
@nan
1996 թուականի Յունիսին հրատարակուած գիտական յօդուած
@hyw
1996 թվականի հունիսին հրատարակված գիտական հոդված
@hy
1996年の論文
@ja
1996年論文
@yue
1996年論文
@zh-hant
1996年論文
@zh-hk
1996年論文
@zh-mo
1996年論文
@zh-tw
1996年论文
@wuu
name
The peroxisome biogenesis diso ...... stability of the PTS1 receptor
@ast
The peroxisome biogenesis diso ...... stability of the PTS1 receptor
@en
The peroxisome biogenesis diso ...... stability of the PTS1 receptor
@en-gb
The peroxisome biogenesis diso ...... stability of the PTS1 receptor
@nl
type
label
The peroxisome biogenesis diso ...... stability of the PTS1 receptor
@ast
The peroxisome biogenesis diso ...... stability of the PTS1 receptor
@en
The peroxisome biogenesis diso ...... stability of the PTS1 receptor
@en-gb
The peroxisome biogenesis diso ...... stability of the PTS1 receptor
@nl
prefLabel
The peroxisome biogenesis diso ...... stability of the PTS1 receptor
@ast
The peroxisome biogenesis diso ...... stability of the PTS1 receptor
@en
The peroxisome biogenesis diso ...... stability of the PTS1 receptor
@en-gb
The peroxisome biogenesis diso ...... stability of the PTS1 receptor
@nl
P2093
P2860
P1433
P1476
The peroxisome biogenesis diso ...... stability of the PTS1 receptor
@en
P2093
J C Morrell
J E Kalish
N Braverman
P2860
P304
P356
10.1002/J.1460-2075.1996.TB00654.X
P407
P577
1996-06-01T00:00:00Z