Peroxisomal Biogenesis Factor 19 (PEX19)

HK33; PMP1; PMPI; PXF; PXMP1; Peroxisomal Farnesylated Protein; Housekeeping Gene,33kD; Peroxin-19

SPN	R092
HGNC	9713
MGI	1334458
PubMed	15007061
UniProt	P40855
Wiki	PEX19

The covalent attachment of prenyl lipids, such as farnesyl or geranylgeranyl groups, by specific transferases is indispensable for the cellular sorting of many proteins.

Human PEX19 expression also restored peroxisomal protein import in fibroblasts from a patient with Zellweger syndrome of CG-J. This patient was found to be homozygous for an inactivating mutation, a 1-bp insertion . These results demonstrated that PEX19 is the causative gene for CG-J PBD and suggested that the C-terminal part of the PEX19 protein, including the CAAX homology box, is required for its biologic function. Moreover, the PEX19 protein is apparently involved in the initial stage of peroxisome membrane assembly, before the import of matrix protein.

Reference

"Sequence of a putative human housekeeping gene (HK33) localized on chromosome 1."
Gene 146:291-295(1994) [PubMed] [Europe PMC] [Abstract]

"Genomic organization and molecular characterization of a gene encoding HsPXF, a human peroxisomal farnesylated protein."
Genomics 45:200-210(1997) [PubMed] [Europe PMC] [Abstract]

"Human PEX19: cDNA cloning by functional complementation, mutation analysis in a patient with Zellweger syndrome, and potential role in peroxisomal membrane assembly."
Proc. Natl. Acad. Sci. U.S.A. 96:2116-2121(1999) [PubMed] [Europe PMC] [Abstract]

"The DNA sequence and biological annotation of human chromosome 1."
Nature 441:315-321(2006) [PubMed] [Europe PMC] [Abstract]

"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]

"Human adrenoleukodystrophy protein and related peroxisomal ABC transporters interact with the peroxisomal assembly protein PEX19p."
Biochem. Biophys. Res. Commun. 271:144-150(2000) [PubMed] [Europe PMC] [Abstract]

"PEX19 binds multiple peroxisomal membrane proteins, is predominantly cytoplasmic, and is required for peroxisome membrane synthesis."
J. Cell Biol. 148:931-944(2000) [PubMed] [Europe PMC] [Abstract]

"Pex19p dampens the p19ARF-p53-p21WAF1 tumor suppressor pathway."
J. Biol. Chem. 276:18649-18652(2001) [PubMed] [Europe PMC] [Abstract]

"Human pex19p binds peroxisomal integral membrane proteins at regions distinct from their sorting sequences."
Mol. Cell. Biol. 21:4413-4424(2001) [PubMed] [Europe PMC] [Abstract]

"Two splice variants of human PEX19 exhibit distinct functions in peroxisomal assembly."
Biochem. Biophys. Res. Commun. 291:1180-1186(2002) [PubMed] [Europe PMC] [Abstract]

"PEX3 functions as a PEX19 docking factor in the import of class I peroxisomal membrane proteins."
J. Cell Biol. 164:863-875(2004) [PubMed] [Europe PMC] [Abstract]

"PEX19 is a predominantly cytosolic chaperone and import receptor for class 1 peroxisomal membrane proteins."
J. Cell Biol. 164:57-67(2004) [PubMed] [Europe PMC] [Abstract]

"A quantitative atlas of mitotic phosphorylation."
Proc. Natl. Acad. Sci. U.S.A. 105:10762-10767(2008) [PubMed] [Europe PMC] [Abstract]

"A mutation in PEX19 causes a severe clinical phenotype in a patient with peroxisomal biogenesis disorder."
Am. J. Med. Genet. A 152:2318-2321(2010) [PubMed] [Europe PMC] [Abstract]

"Initial characterization of the human central proteome."
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]

"N-terminal acetylome analyses and functional insights of the N-terminal acetyltransferase NatB."
Proc. Natl. Acad. Sci. U.S.A. 109:12449-12454(2012) [PubMed] [Europe PMC] [Abstract]

"An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]

"Structural basis for competitive interactions of Pex14 with the import receptors Pex5 and Pex19."
EMBO J. 28:745-754(2009) [PubMed] [Europe PMC] [Abstract]

"Structural basis for docking of peroxisomal membrane protein carrier Pex19p onto its receptor Pex3p."
EMBO J. 29:4083-4093(2010) [PubMed] [Europe PMC] [Abstract]

"Insights into peroxisome function from the structure of PEX3 in complex with a soluble fragment of PEX19."
J. Biol. Chem. 285:25410-25417(2010) [PubMed] [Europe PMC] [Abstract]