Differential protein import deficiencies in human peroxisome assembly disorders.
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Topogenesis of peroxisomal membrane protein requires a short, positively charged intervening-loop sequence and flanking hydrophobic segments. study using human membrane protein PMP34Alkyl-dihydroxyacetonephosphate synthase. Fate in peroxisome biogenesis disorders and identification of the point mutation underlying a single enzyme deficiencyHuman PEX7 encodes the peroxisomal PTS2 receptor and is responsible for rhizomelic chondrodysplasia punctataRhizomelic chondrodysplasia punctata is a peroxisomal protein targeting disease caused by a non-functional PTS2 receptorRhizomelic chondrodysplasia punctata is caused by deficiency of human PEX7, a homologue of the yeast PTS2 receptorIsolation of the human PEX12 gene, mutated in group 3 of the peroxisome biogenesis disordersMutations in the PTS1 receptor gene, PXR1, define complementation group 2 of the peroxisome biogenesis disordersIdentification of PAHX, a Refsum disease genePEX12 encodes an integral membrane protein of peroxisomesThe peroxisome biogenesis disorder group 4 gene, PXAAA1, encodes a cytoplasmic ATPase required for stability of the PTS1 receptorClofibrate-inducible, 28-kDa peroxisomal integral membrane protein is encoded by PEX11PEX12, the pathogenic gene of group III Zellweger syndrome: cDNA cloning by functional complementation on a CHO cell mutant, patient analysis, and characterization of PEX12pPeroxisome targeting signal type 1 (PTS1) receptor is involved in import of both PTS1 and PTS2: studies with PEX5-defective CHO cell mutants.The peroxin pex3p initiates membrane assembly in peroxisome biogenesisHuman peroxisomal targeting signal-1 receptor restores peroxisomal protein import in cells from patients with fatal peroxisomal disordersDisruption 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 receptorPex17p of Saccharomyces cerevisiae is a novel peroxin and component of the peroxisomal protein translocation machineryReevaluation of the role of Pex1 and dynamin-related proteins in peroxisome membrane biogenesisThe SH3 domain of the Saccharomyces cerevisiae peroxisomal membrane protein Pex13p functions as a docking site for Pex5p, a mobile receptor for the import PTS1-containing proteins.Peb1p (Pas7p) is an intraperoxisomal receptor for the NH2-terminal, type 2, peroxisomal targeting sequence of thiolase: Peb1p itself is targeted to peroxisomes by an NH2-terminal peptide.The import receptor for the peroxisomal targeting signal 2 (PTS2) in Saccharomyces cerevisiae is encoded by the PAS7 gene.Lpx1p is a peroxisomal lipase required for normal peroxisome morphology.PAS7 encodes a novel yeast member of the WD-40 protein family essential for import of 3-oxoacyl-CoA thiolase, a PTS2-containing protein, into peroxisomesSaccharomyces cerevisiae pex3p and pex19p are required for proper localization and stability of peroxisomal membrane proteinsPEB1 (PAS7) in Saccharomyces cerevisiae encodes a hydrophilic, intra-peroxisomal protein that is a member of the WD repeat family and is essential for the import of thiolase into peroxisomes.Localization and targeting of isocitrate lyases in Saccharomyces cerevisiae.A mouse model for Zellweger syndromeAlkyl-dihydroxyacetone phosphate synthase and dihydroxyacetone phosphate acyltransferase form a protein complex in peroxisomesTopogenesis and homeostasis of fatty acyl-CoA reductase 1Involvement of the endoplasmic reticulum in peroxisome formationCharacterization of a novel component of the peroxisomal protein import apparatus using fluorescent peroxisomal proteinsPay32p of the yeast Yarrowia lipolytica is an intraperoxisomal component of the matrix protein translocation machineryTargeted deletion of the PEX2 peroxisome assembly gene in mice provides a model for Zellweger syndrome, a human neuronal migration disorder.A mobile PTS2 receptor for peroxisomal protein import in Pichia pastoris.Formation of the peroxisome lumen is abolished by loss of Pichia pastoris Pas7p, a zinc-binding integral membrane protein of the peroxisome.The Pichia pastoris PER6 gene product is a peroxisomal integral membrane protein essential for peroxisome biogenesis and has sequence similarity to the Zellweger syndrome protein PAF-1.Oxidation of pristanic acid in fibroblasts and its application to the diagnosis of peroxisomal beta-oxidation defectsThe Pichia pastoris peroxisomal protein PAS8p is the receptor for the C-terminal tripeptide peroxisomal targeting signalMutational spectrum in the PEX7 gene and functional analysis of mutant alleles in 78 patients with rhizomelic chondrodysplasia punctata type 1.
P2860
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P2860
Differential protein import deficiencies in human peroxisome assembly disorders.
description
1994 nî lūn-bûn
@nan
1994年の論文
@ja
1994年論文
@yue
1994年論文
@zh-hant
1994年論文
@zh-hk
1994年論文
@zh-mo
1994年論文
@zh-tw
1994年论文
@wuu
1994年论文
@zh
1994年论文
@zh-cn
name
Differential protein import deficiencies in human peroxisome assembly disorders.
@en
type
label
Differential protein import deficiencies in human peroxisome assembly disorders.
@en
prefLabel
Differential protein import deficiencies in human peroxisome assembly disorders.
@en
P2093
P2860
P356
P1476
Differential protein import deficiencies in human peroxisome assembly disorders.
@en
P2093
P2860
P304
P356
10.1083/JCB.125.4.755
P407
P577
1994-05-01T00:00:00Z