Human peroxisomal targeting signal-1 receptor restores peroxisomal protein import in cells from patients with fatal peroxisomal disorders
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Mutation in PEX16 is causal in the peroxisome-deficient Zellweger syndrome of complementation group D.Identification and characterization of the human orthologue of yeast Pex14pA novel human DNA-binding protein with sequence similarity to a subfamily of redox proteins which is able to repress RNA-polymerase-III-driven transcription of the Alu-family retroposons in vitroIdentification of peroxisomal proteins by using M13 phage protein VI phage display: molecular evidence that mammalian peroxisomes contain a 2,4-dienoyl-CoA reductaseCharacterization of human and murine PMP20 peroxisomal proteins that exhibit antioxidant activity in vitroPEX12 interacts with PEX5 and PEX10 and acts downstream of receptor docking in peroxisomal matrix protein importIdentification of PEX5p-related novel peroxisome-targeting signal 1 (PTS1)-binding proteins in mammalsHuman 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 receptorIdentification of a human PTS1 receptor docking protein directly required for peroxisomal protein importPex13p is an SH3 protein of the peroxisome membrane and a docking factor for the predominantly cytoplasmic PTs1 receptorPEX3 functions as a PEX19 docking factor in the import of class I peroxisomal membrane proteinsProtein quaternary structure and expression levels contribute to peroxisomal-targeting-sequence-1-mediated peroxisomal import of human soluble epoxide hydrolaseIdentification of PEX10, the gene defective in complementation group 7 of the peroxisome-biogenesis disordersThe 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 PEX12pHsp70 regulates the interaction between the peroxisome targeting signal type 1 (PTS1)-receptor Pex5p and PTS1Peroxisome targeting signal type 1 (PTS1) receptor is involved in import of both PTS1 and PTS2: studies with PEX5-defective CHO cell mutants.Peroxisomal targeting signal receptor Pex5p interacts with cargoes and import machinery components in a spatiotemporally differentiated manner: conserved Pex5p WXXXF/Y motifs are critical for matrix protein import.Human 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 factorsMultiple 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 receptorThe similarity between N-terminal targeting signals for protein import into different organelles and its evolutionary relevanceThe 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.Saccharomyces cerevisiae PTS1 receptor Pex5p interacts with the SH3 domain of the peroxisomal membrane protein Pex13p in an unconventional, non-PXXP-related manner.Saccharomyces cerevisiae acyl-CoA oxidase follows a novel, non-PTS1, import pathway into peroxisomes that is dependent on Pex5p.The cytosolic DnaJ-like protein djp1p is involved specifically in peroxisomal protein import.The import receptor for the peroxisomal targeting signal 2 (PTS2) in Saccharomyces cerevisiae is encoded by the PAS7 gene.Identification of Pex13p a peroxisomal membrane receptor for the PTS1 recognition factorRecognition of peroxisomal targeting signal type 1 by the import receptor Pex5p.Membrane association of the cycling peroxisome import receptor Pex5p.A mouse model for Zellweger syndromeCharacterization of peroxisomal Pex5p from rat liver. Pex5p in the Pex5p-Pex14p membrane complex is a transmembrane protein.Genomic structure and identification of 11 novel mutations of the PEX6 (peroxisome assembly factor-2) gene in patients with peroxisome biogenesis disorders.Peroxisomal remnant structures in Hansenula polymorpha Pex5 cells can develop into normal peroxisomes upon induction of the PTS2 protein amine oxidase.Saccharomyces cerevisiae Pex14p contains two independent Pex5p binding sites, which are both essential for PTS1 protein import.Peroxisome biogenesis and peroxisome biogenesis disorders.Pay32p of the yeast Yarrowia lipolytica is an intraperoxisomal component of the matrix protein translocation machinery
P2860
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P2860
Human peroxisomal targeting signal-1 receptor restores peroxisomal protein import in cells from patients with fatal peroxisomal disorders
description
1995 nî lūn-bûn
@nan
1995 թուականի Յուլիսին հրատարակուած գիտական յօդուած
@hyw
1995 թվականի հուլիսին հրատարակված գիտական հոդված
@hy
1995年の論文
@ja
1995年論文
@yue
1995年論文
@zh-hant
1995年論文
@zh-hk
1995年論文
@zh-mo
1995年論文
@zh-tw
1995年论文
@wuu
name
Human peroxisomal targeting si ...... th fatal peroxisomal disorders
@ast
Human peroxisomal targeting si ...... th fatal peroxisomal disorders
@en
Human peroxisomal targeting si ...... th fatal peroxisomal disorders
@nl
type
label
Human peroxisomal targeting si ...... th fatal peroxisomal disorders
@ast
Human peroxisomal targeting si ...... th fatal peroxisomal disorders
@en
Human peroxisomal targeting si ...... th fatal peroxisomal disorders
@nl
prefLabel
Human peroxisomal targeting si ...... th fatal peroxisomal disorders
@ast
Human peroxisomal targeting si ...... th fatal peroxisomal disorders
@en
Human peroxisomal targeting si ...... th fatal peroxisomal disorders
@nl
P2093
P2860
P356
P1476
Human peroxisomal targeting si ...... th fatal peroxisomal disorders
@en
P2093
B L Bertolaet
E A Wiemer
K R Johnson
M J Wheelock
S Subramani
W M Nuttley
P2860
P356
10.1083/JCB.130.1.51
P407
P577
1995-07-01T00:00:00Z