Human PEX1 cloned by functional complementation on a CHO cell mutant is responsible for peroxisome-deficient Zellweger syndrome of complementation group I - Test2 - elhamkhani - TriplyDB

Human PEX1 cloned by functional complementation on a CHO cell mutant is responsible for peroxisome-deficient Zellweger syndrome of complementation group I

Human PEX1 cloned by functional complementation on a CHO cell mutant is responsible for peroxisome-deficient Zellweger syndrome of complementation group I

http://www.wikidata.org/entity/Q24645143

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Mutation in PEX16 is causal in the peroxisome-deficient Zellweger syndrome of complementation group D.Human PEX19: cDNA cloning by functional complementation, mutation analysis in a patient with Zellweger syndrome, and potential role in peroxisomal membrane assembly Topogenesis of peroxisomal membrane protein requires a short, positively charged intervening-loop sequence and flanking hydrophobic segments. study using human membrane protein PMP34 Phenotype-genotype relationships in peroxisome biogenesis disorders of PEX1-defective complementation group 1 are defined by Pex1p-Pex6p interaction Mutations in the peroxin Pex26p responsible for peroxisome biogenesis disorders of complementation group 8 impair its stability, peroxisomal localization, and interaction with the Pex1p x Pex6p complex In vitro transport of membrane proteins to peroxisomes by shuttling receptor Pex19p Posttranslational regulation of fatty acyl-CoA reductase 1, Far1, controls ether glycerophospholipid synthesis Dynamic and functional assembly of the AAA peroxins, Pex1p and Pex6p, and their membrane receptor Pex26p Characterization of the interaction between recombinant human peroxin Pex3p and Pex19p: identification of TRP-104 IN Pex3p as a critical residue for the interaction Clofibrate-inducible, 28-kDa peroxisomal integral membrane protein is encoded by PEX11 PEX12, the pathogenic gene of group III Zellweger syndrome: cDNA cloning by functional complementation on a CHO cell mutant, patient analysis, and characterization of PEX12p Mutations in novel peroxin gene PEX26 that cause peroxisome-biogenesis disorders of complementation group 8 provide a genotype-phenotype correlation PEX3 is the causal gene responsible for peroxisome membrane assembly-defective Zellweger syndrome of complementation group G.The peroxin pex3p initiates membrane assembly in peroxisome biogenesis Crystal structure of the conserved N-terminal domain of the peroxisomal matrix protein import receptor, Pex14p The peroxin Pex14p. cDNA cloning by functional complementation on a Chinese hamster ovary cell mutant, characterization, and functional analysis Catalase-less peroxisomes. Implication in the milder forms of peroxisome biogenesis disorder PEX1 mutations in complementation group 1 of Zellweger spectrum patients correlate with severity of disease Novel PEX1 mutations and genotype-phenotype correlations in Australasian peroxisome biogenesis disorder patients Cysteine ubiquitination of PTS1 receptor Pex5p regulates Pex5p recycling.Genomic structure and identification of 11 novel mutations of the PEX6 (peroxisome assembly factor-2) gene in patients with peroxisome biogenesis disorders.Regulation of somatostatin receptor 4-mediated cytostatic effects by CD26 in malignant pleural mesothelioma Peroxisome biogenesis and peroxisome biogenesis disorders.Characterization of two common 5' polymorphisms in PEX1 and correlation to survival in PEX1 peroxisome biogenesis disorder patients.Shuttling mechanism of peroxisome targeting signal type 1 receptor Pex5: ATP-independent import and ATP-dependent export.Deletion of a single allele of the Pex11β gene is sufficient to cause oxidative stress, delayed differentiation and neuronal death in mouse brain Peroxisome biogenesis and human peroxisome-deficiency disorders.Distinct modes of ubiquitination of peroxisome-targeting signal type 1 (PTS1) receptor Pex5p regulate PTS1 protein import.Intracellular localization, function, and dysfunction of the peroxisome-targeting signal type 2 receptor, Pex7p, in mammalian cells.Disruption of the interaction of the longer isoform of Pex5p, Pex5pL, with Pex7p abolishes peroxisome targeting signal type 2 protein import in mammals. Study with a novel Pex5-impaired Chinese hamster ovary cell mutant.Newly identified Chinese hamster ovary cell mutants are defective in biogenesis of peroxisomal membrane vesicles (Peroxisomal ghosts), representing a novel complementation group in mammals.Disorders of peroxisome biogenesis due to mutations in PEX1: phenotypes and PEX1 protein levels.Fusion of small peroxisomal vesicles in vitro reconstructs an early step in the in vivo multistep peroxisome assembly pathway of Yarrowia lipolytica.Pex5p stabilizes Pex14p: a study using a newly isolated pex5 CHO cell mutant, ZPEG101.Characterization of the peroxisomal cycling receptor, Pex5p, using a cell-free in vitro import system.Hansenula polymorpha Pex1p and Pex6p are peroxisome-associated AAA proteins that functionally and physically interact.Pex5p imports folded tetrameric catalase by interaction with Pex13p.Recruiting mechanism of the AAA peroxins, Pex1p and Pex6p, to Pex26p on the peroxisomal membrane.PEX1:PEX6:PEX26:ZFAND6:Ub:PEX5S;L:PEX14:PEX13:PEX2:PEX10:PEX12 dissociates yielding cytosolic Ub:PEX5S;L and membrane PEX14:PEX13:PEX2:PEX10:PEX12 PEX1:PEX6:PEX26:ZFAND6 dissociates Ub:PEX5L and PEX7 from PEX14:PEX13:PEX2:PEX10:PEX12 and translocates PEX5L and PEX7 from the peroxisomal membrane to the cytosol

P2860

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P2860

Human PEX1 cloned by functional complementation on a CHO cell mutant is responsible for peroxisome-deficient Zellweger syndrome of complementation group I

http://www.wikidata.org/entity/Q24645143

description

1998 nî lūn-bûn

@nan

1998 թուականի Ապրիլին հրատարակուած գիտական յօդուած

@hyw

1998 թվականի ապրիլին հրատարակված գիտական հոդված

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1998年の論文

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1998年論文

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1998年論文

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1998年論文

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1998年論文

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1998年論文

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1998年论文

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name

Human PEX1 cloned by functiona ...... ome of complementation group I

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Human PEX1 cloned by functiona ...... ome of complementation group I

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Human PEX1 cloned by functiona ...... ome of complementation group I

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Human PEX1 cloned by functiona ...... ome of complementation group I

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Human PEX1 cloned by functiona ...... ome of complementation group I

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Human PEX1 cloned by functiona ...... ome of complementation group I

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Human PEX1 cloned by functiona ...... ome of complementation group I

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Human PEX1 cloned by functiona ...... ome of complementation group I

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Human PEX1 cloned by functiona ...... ome of complementation group I

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P2860

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P1433

Proceedings of the National Academy of Sciences of the United States of America

P1476

Human PEX1 cloned by functiona ...... ome of complementation group I

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P2093

K Okumoto

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N Shimozawa

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R Toyama

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T Tsukamoto

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Y Fujiki

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Y Suzuki

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P2860

A human gene responsible for Zellweger syndrome that affects peroxisome assembly

Human PEX7 encodes the peroxisomal PTS2 receptor and is responsible for rhizomelic chondrodysplasia punctata

Rhizomelic chondrodysplasia punctata is a peroxisomal protein targeting disease caused by a non-functional PTS2 receptor

Rhizomelic chondrodysplasia punctata is caused by deficiency of human PEX7, a homologue of the yeast PTS2 receptor

Isolation of the human PEX12 gene, mutated in group 3 of the peroxisome biogenesis disorders

Mutations in PEX1 are the most common cause of peroxisome biogenesis disorders

Mutations in the PTS1 receptor gene, PXR1, define complementation group 2 of the peroxisome biogenesis disorders

Human peroxisome assembly factor-2 (PAF-2): a gene responsible for group C peroxisome biogenesis disorder in humans

PEX12 encodes an integral membrane protein of peroxisomes

The peroxisome biogenesis disorder group 4 gene, PXAAA1, encodes a cytoplasmic ATPase required for stability of the PTS1 receptor

P304

4350-5

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P31

scholarly article

P356

10.1073/PNAS.95.8.4350

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P433

8

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P478

95

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P577

1998-04-14T00:00:00Z

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P5875

51331687

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P698

9539740

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P932

22492

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