A human gene responsible for Zellweger syndrome that affects peroxisome assembly
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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 peroxisomesHuman PEX19: cDNA cloning by functional complementation, mutation analysis in a patient with Zellweger syndrome, and potential role in peroxisomal membrane assemblyPEX12 interacts with PEX5 and PEX10 and acts downstream of receptor docking in peroxisomal matrix protein importMolecular anatomy of the peroxin Pex12p: ring finger domain is essential for Pex12p function and interacts with the peroxisome-targeting signal type 1-receptor Pex5p and a ring peroxin, Pex10pPhenotype-genotype relationships in peroxisome biogenesis disorders of PEX1-defective complementation group 1 are defined by Pex1p-Pex6p interactionThe TRIM37 gene encodes a peroxisomal RING-B-box-coiled-coil protein: classification of mulibrey nanism as a new peroxisomal disorderProteomic analysis of rat liver peroxisome: presence of peroxisome-specific isozyme of Lon proteaseCloning and characterization of the gene encoding the human peroxisomal assembly protein Pex3pPex13p is an SH3 protein of the peroxisome membrane and a docking factor for the predominantly cytoplasmic PTs1 receptorHuman peroxisome assembly factor-2 (PAF-2): a gene responsible for group C peroxisome biogenesis disorder in humansClofibrate-inducible, 28-kDa peroxisomal integral membrane protein is encoded by PEX11The glomerulosclerosis gene Mpv17 encodes a peroxisomal protein producing reactive oxygen speciesPEX12, 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.Phenotype-genotype relationships in complementation group 3 of the peroxisome-biogenesis disordersPEX13 is mutated in complementation group 13 of the peroxisome-biogenesis disorders.A close relative of the adrenoleukodystrophy (ALD) gene codes for a peroxisomal protein with a specific expression patternLarge deletion of the peroxisomal acyl-CoA oxidase gene in pseudoneonatal adrenoleukodystrophyHuman PEX1 cloned by functional complementation on a CHO cell mutant is responsible for peroxisome-deficient Zellweger syndrome of complementation group IAn apoptosis-inhibiting gene from a nuclear polyhedrosis virus encoding a polypeptide with Cys/His sequence motifsFunctional identification of a Leishmania gene related to the peroxin 2 gene reveals common ancestry of glycosomes and peroxisomesA unified nomenclature for peroxisome biogenesis factorsHuman peroxisomal targeting signal-1 receptor restores peroxisomal protein import in cells from patients with fatal peroxisomal disordersMultiple 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 functionPeroxisome biogenesis in mammalian cellsThe Pex1-G844D mouse: a model for mild human Zellweger spectrum disorder.Giant peroxisomes in oleic acid-induced Saccharomyces cerevisiae lacking the peroxisomal membrane protein Pmp27p.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.Disruption of the Saccharomyces cerevisiae FAT1 gene decreases very long-chain fatty acyl-CoA synthetase activity and elevates intracellular very long-chain fatty acid concentrations.Pex2 and pex12 function as protein-ubiquitin ligases in peroxisomal protein import.Identification and characterization of the putative human peroxisomal C-terminal targeting signal import receptorThe peroxin Pex14p. cDNA cloning by functional complementation on a Chinese hamster ovary cell mutant, characterization, and functional analysisCatalase-less peroxisomes. Implication in the milder forms of peroxisome biogenesis disorderRing finger in the peroxisome assembly factor-1Molecular mechanism of a temperature-sensitive phenotype in peroxisomal biogenesis disorderCorrection by gene expression of biochemical abnormalities in fibroblasts from Zellweger patientsMembrane topology of the 22 kDa integral peroxisomal membrane proteinPeroxisome assembly factor 1: nonsense mutation in a peroxisome-deficient Chinese hamster ovary cell mutant and deletion analysis
P2860
Q22008461-93E7D8EB-D2A9-4ADD-9939-CFE20E865C08Q22008726-E7647A76-0CD0-4CD0-A5C5-095D07E3DD17Q22008838-32EFEA8E-0D36-43A3-BA3C-B2669BF3FCF7Q22010795-D99C6C0D-A243-441D-BD2C-BC4EFCCDA2F5Q22254195-DC127CDD-50E3-4942-8779-49D72B6C1351Q24291411-E6F1FC5B-B10F-4C2C-A455-5B22E7D64A74Q24293462-99B8B9EE-B33F-4BC1-973F-A13EBDDE05BCQ24297876-3727A4F9-580F-402A-8F95-9EA729BC044FQ24313158-8FF566C4-9DFA-4EBA-9475-D97F963E8352Q24313219-BD0A7C33-4167-49B7-A0F5-8004A97BD5ACQ24323096-AD257CCE-47D4-427F-81DA-78809300233FQ24336400-27C6CA79-0B8E-4503-BA5B-6702473CF1D5Q24336548-6E9D91EF-B549-469D-BE00-A4F205D48300Q24522572-3365D203-678E-4F72-80E3-1F3922945CA0Q24533793-D578874E-ECE2-49FA-95CC-89EEE3F49BB7Q24539414-40CC281A-A9C7-4687-B434-804F2697AE6FQ24540126-BEBD294D-98A2-49D9-B6F8-483D350089D6Q24567552-68937088-4777-4D49-9B7C-05C0B3AF673AQ24628708-54A27FF1-2F14-4FA0-ADF1-9FE2A77F9402Q24645143-51909E1A-34B2-426E-A0F4-288B705418DCQ24646704-195AAD9E-EC91-4488-B42B-9F632DDCEFE3Q24647237-33CE2A7E-F97B-437C-8F9C-02DDD3EB295DQ24671250-C5FF2FBA-4047-47DD-8677-D1C41EE244A3Q24672527-BB928B9C-0140-438A-95CE-C51A4DF43C2DQ24681727-0352E140-6818-46CA-97D0-A60BDF971189Q26774697-42AD8090-0D2C-4926-A2B4-F2BDE74002F8Q26859014-30184C98-E275-47C9-95E8-BA22F3B30C57Q27302267-7EF3831C-5E02-47EF-8389-2A30E9A18050Q27929757-37CFE8EE-373F-4EF7-8136-CDF8545AE382Q27930382-FDCE7E23-8D63-458C-B959-F148DD2D26FDQ27932646-81546507-2E72-4F0E-8C9E-C25C37D119E8Q27934783-E1605DF5-A9E3-44C4-B37A-2F4AC8B2E5D8Q28115941-1CD5D378-D763-4EFA-8E0D-76894B1BDF53Q28142062-33F40BB0-1041-4A38-8177-2AD395B43B29Q28144608-75E5959C-4F06-4E94-9F6A-4B5EC32404B9Q28201641-07E58969-74CF-4438-A4ED-31815F042843Q28260700-B6685185-AB99-4FB8-AE1E-9D37BC86B0D8Q28285599-0E849F63-7BCF-4657-BC48-19EAC7FB3991Q28569095-3F94074A-638B-4490-8AAA-32C46BC12E84Q28577791-A75A3F0F-A013-42DF-A349-45E71AD2FFB0
P2860
A human gene responsible for Zellweger syndrome that affects peroxisome assembly
description
1992 nî lūn-bûn
@nan
1992 թուականի Փետրուարին հրատարակուած գիտական յօդուած
@hyw
1992 թվականի փետրվարին հրատարակված գիտական հոդված
@hy
1992年の論文
@ja
1992年論文
@yue
1992年論文
@zh-hant
1992年論文
@zh-hk
1992年論文
@zh-mo
1992年論文
@zh-tw
1992年论文
@wuu
name
A human gene responsible for Zellweger syndrome that affects peroxisome assembly
@ast
A human gene responsible for Zellweger syndrome that affects peroxisome assembly
@en
A human gene responsible for Zellweger syndrome that affects peroxisome assembly
@en-gb
A human gene responsible for Zellweger syndrome that affects peroxisome assembly
@nl
type
label
A human gene responsible for Zellweger syndrome that affects peroxisome assembly
@ast
A human gene responsible for Zellweger syndrome that affects peroxisome assembly
@en
A human gene responsible for Zellweger syndrome that affects peroxisome assembly
@en-gb
A human gene responsible for Zellweger syndrome that affects peroxisome assembly
@nl
prefLabel
A human gene responsible for Zellweger syndrome that affects peroxisome assembly
@ast
A human gene responsible for Zellweger syndrome that affects peroxisome assembly
@en
A human gene responsible for Zellweger syndrome that affects peroxisome assembly
@en-gb
A human gene responsible for Zellweger syndrome that affects peroxisome assembly
@nl
P2093
P356
P1433
P1476
A human gene responsible for Zellweger syndrome that affects peroxisome assembly
@en
P2093
N Shimozawa
T Tsukamoto
Y Shirayoshi
P304
P356
10.1126/SCIENCE.1546315
P407
P577
1992-02-28T00:00:00Z