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
about
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 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, Pex10pTopogenesis of peroxisomal membrane protein requires a short, positively charged intervening-loop sequence and flanking hydrophobic segments. study using human membrane protein PMP34Two proteases, trypsin domain-containing 1 (Tysnd1) and peroxisomal lon protease (PsLon), cooperatively regulate fatty acid β-oxidation in peroxisomal matrixDynamic and functional assembly of the AAA peroxins, Pex1p and Pex6p, and their membrane receptor Pex26pThe membrane biogenesis peroxin Pex16p. Topogenesis and functional roles in peroxisomal membrane assemblyThe peroxisomal membrane protein import receptor Pex3p is directly transported to peroxisomes by a novel Pex19p- and Pex16p-dependent pathwayHsp70 regulates the interaction between the peroxisome targeting signal type 1 (PTS1)-receptor Pex5p and PTS1Phenotype-genotype relationships in complementation group 3 of the peroxisome-biogenesis disordersPEX13 is mutated in complementation group 13 of the peroxisome-biogenesis disorders.The peroxin pex3p initiates membrane assembly in peroxisome biogenesisThe peroxin Pex14p. cDNA cloning by functional complementation on a Chinese hamster ovary cell mutant, characterization, and functional analysisThe pathogenic peroxin Pex26p recruits the Pex1p-Pex6p AAA ATPase complexes to peroxisomesNovel mutations in the PEX12 gene of patients with a peroxisome biogenesis disorderAWP1/ZFAND6 functions in Pex5 export by interacting with cys-monoubiquitinated Pex5 and Pex6 AAA ATPaseCharacterization of the mammalian peroxisomal import machinery: Pex2p, Pex5p, Pex12p, and Pex14p are subunits of the same protein assemblyCysteine ubiquitination of PTS1 receptor Pex5p regulates Pex5p recycling.A novel human striated muscle RING zinc finger protein, SMRZ, interacts with SMT3b via its RING domain.Peroxisome biogenesis and peroxisome biogenesis disorders.Characterization of glycosomal RING finger proteins of trypanosomatids.Unique requirements for mono- and polyubiquitination of the peroxisomal targeting signal co-receptor, Pex20.Peroxisome biogenesis and human peroxisome-deficiency disorders.Mild reduction of plasmalogens causes rhizomelic chondrodysplasia punctata: functional characterization of a novel mutation.Distinct modes of ubiquitination of peroxisome-targeting signal type 1 (PTS1) receptor Pex5p regulate PTS1 protein import.Genetic classification and mutational spectrum of more than 600 patients with a Zellweger syndrome spectrum disorder.Molecular mechanisms of import of peroxisome-targeting signal type 2 (PTS2) proteins by PTS2 receptor Pex7p and PTS1 receptor Pex5pL.Stress induces peroxisome biogenesis genes.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.Mff functions with Pex11pβ and DLP1 in peroxisomal fissionIsolation and characterization of novel phenotype CHO cell mutants defective in peroxisome assembly, using ICR191 as a potent mutagenic agent.Whole-exome sequencing in patients with inherited neuropathies: outcome and challenges.System to quantify the import of peroxisomal matrix proteins by fluorescence intensity.Peroxisome biogenesis: a novel inducible PEX19 splicing variant is involved in early stages of peroxisome proliferation.Peroxin Pex14p is the key component for coordinated autophagic degradation of mammalian peroxisomes by direct binding to LC3-II.
P2860
Q22008461-B786FE43-993A-4C3C-A1A4-30F10EF86909Q22008838-57498380-8A11-4719-BE6D-25BB9CE0BEA2Q22010795-BAE0DD78-AA38-413F-9ADE-D122FF203D90Q22254195-764BEBE3-34BE-4263-B7DB-866629468BE3Q24290647-238FB79C-1D25-4622-B9BE-A1C5B9086CBCQ24293436-1237EDE1-D3C9-46DE-B3F8-CF96EBCB22A0Q24297548-E3DED0F9-F118-4329-9516-96D7A6DD6903Q24306509-158EC543-5646-4C1F-9FAB-3A9B75D2FE11Q24311655-4C49AD03-2A4A-4CEC-82B0-2DF1F1C76094Q24533332-34A8CAD2-3770-4B1D-90C6-F0166DA950A9Q24539414-17CEB4FA-242D-4CF3-B459-02E00129E38FQ24540126-8B35CBC2-8F52-4C29-A8E2-EB35EF0649C6Q24549973-AFAF24A7-F44F-4012-BBC9-2BA994679D64Q28142062-FB4D63B0-87D0-4F13-878F-60F9DEE73320Q28201464-A03BAEA3-166E-4699-8789-4B5F6CE6864BQ28211700-57F511F7-2CB7-4FA9-9A3B-FA66E6737A4DQ28513412-0B6C9066-C799-4A47-B2B3-1247F91C20F3Q28579082-DCD760A2-0997-4486-BD26-1FE133E75B80Q29871525-AC02F247-B2BD-4267-8B9D-4309232C5A89Q33941031-FD2A712F-0515-41B6-A1DD-FCB3C2FF84CDQ33957100-4A1BBBD8-7FEA-4B9E-9A79-33E12D3CB05CQ35990056-61C0B58D-F173-4BE9-932C-1EF7CE0021C0Q36666245-A96F7068-86BD-4408-8AD4-C8414E8221FFQ37670278-6063C706-5D2D-4408-8090-AFCFAAD8BE64Q38992882-E2BE1BF2-A70B-4EFC-A050-8DEF0D6450B0Q39012365-6DC92B3C-FA2B-4828-B651-44BDEDF5A47AQ39638543-95568F19-A418-422D-9BB5-DDB91E46A5C8Q40220398-F5B75801-CCFF-42B9-A549-860891BC667CQ40388461-1F5D03C0-E8DB-48F0-8266-CE1A3429E658Q40760857-627E36D7-1AF7-4488-BCDF-2A05A32BC71EQ40886599-5211001C-4E46-4D6A-97B3-D2A576889EF7Q41011005-16FAD540-9B7B-487D-94E3-229F59A9911EQ42125012-D0A77A5A-555E-4A6C-B530-AD3FEE4199C4Q42809122-2A56FB78-A531-4C5E-AD8C-86109A0FC4EBQ44303948-B6DEF6E1-D94A-4588-B2FF-7713D3D7712AQ50481949-7D621C23-C327-4F81-9F3D-2442838186ECQ51066118-5D662C1B-2EA9-4A6C-8C8B-BC829F7355BCQ51746150-8B70AC35-5586-49E5-A2D5-F85613C86BA5
P2860
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
description
1998 nî lūn-bûn
@nan
1998 թուականի Յուլիսին հրատարակուած գիտական յօդուած
@hyw
1998 թվականի հուլիսին հրատարակված գիտական հոդված
@hy
1998年の論文
@ja
1998年論文
@yue
1998年論文
@zh-hant
1998年論文
@zh-hk
1998年論文
@zh-mo
1998年論文
@zh-tw
1998年论文
@wuu
name
PEX12, the pathogenic gene of ...... and characterization of PEX12p
@ast
PEX12, the pathogenic gene of ...... and characterization of PEX12p
@en
PEX12, the pathogenic gene of ...... and characterization of PEX12p
@en-gb
PEX12, the pathogenic gene of ...... and characterization of PEX12p
@nl
type
label
PEX12, the pathogenic gene of ...... and characterization of PEX12p
@ast
PEX12, the pathogenic gene of ...... and characterization of PEX12p
@en
PEX12, the pathogenic gene of ...... and characterization of PEX12p
@en-gb
PEX12, the pathogenic gene of ...... and characterization of PEX12p
@nl
prefLabel
PEX12, the pathogenic gene of ...... and characterization of PEX12p
@ast
PEX12, the pathogenic gene of ...... and characterization of PEX12p
@en
PEX12, the pathogenic gene of ...... and characterization of PEX12p
@en-gb
PEX12, the pathogenic gene of ...... and characterization of PEX12p
@nl
P2093
P2860
P356
P1476
PEX12, the pathogenic gene of ...... and characterization of PEX12p
@en
P2093
N Shimozawa
R J Wanders
T Tsukamoto
P2860
P304
P356
10.1128/MCB.18.7.4324
P407
P577
1998-07-01T00:00:00Z