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Peroxisomal Biogenesis Factor 19 (PEX19)

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

Peroxisomal Biogenesis Factor 19 (PEX19)
CCC E733
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.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPE733Hu01 Recombinant Peroxisomal Biogenesis Factor 19 (PEX19) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies n/a Monoclonal Antibody to Peroxisomal Biogenesis Factor 19 (PEX19) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Peroxisomal Biogenesis Factor 19 (PEX19) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Peroxisomal Biogenesis Factor 19 (PEX19) CLIA Kit Customized Service Offer
n/a ELISA Kit for Peroxisomal Biogenesis Factor 19 (PEX19) ELISA Kit Customized Service Offer

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPE733Mu01 Recombinant Peroxisomal Biogenesis Factor 19 (PEX19) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies n/a Monoclonal Antibody to Peroxisomal Biogenesis Factor 19 (PEX19) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Peroxisomal Biogenesis Factor 19 (PEX19) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Peroxisomal Biogenesis Factor 19 (PEX19) CLIA Kit Customized Service Offer
n/a ELISA Kit for Peroxisomal Biogenesis Factor 19 (PEX19) ELISA Kit Customized Service Offer
  1. "Sequence of a putative human housekeeping gene (HK33) localized on chromosome 1."Gene 146:291-295(1994) [PubMed] [Europe PMC] [Abstract]
  2. "Genomic organization and molecular characterization of a gene encoding HsPXF, a human peroxisomal farnesylated protein."Genomics 45:200-210(1997) [PubMed] [Europe PMC] [Abstract]
  3. "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]
  4. "The DNA sequence and biological annotation of human chromosome 1." Nature 441:315-321(2006) [PubMed] [Europe PMC] [Abstract]
  5. "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]
  6. "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]
  7. "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]
  8. "Pex19p dampens the p19ARF-p53-p21WAF1 tumor suppressor pathway."J. Biol. Chem. 276:18649-18652(2001) [PubMed] [Europe PMC] [Abstract]
  9. "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]
  10. "Two splice variants of human PEX19 exhibit distinct functions in peroxisomal assembly."Biochem. Biophys. Res. Commun. 291:1180-1186(2002) [PubMed] [Europe PMC] [Abstract]
  11. "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]
  12. "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]
  13. "A quantitative atlas of mitotic phosphorylation."Proc. Natl. Acad. Sci. U.S.A. 105:10762-10767(2008) [PubMed] [Europe PMC] [Abstract]
  14. "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]
  15. "Initial characterization of the human central proteome."BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
  16. "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]
  17. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
  18. "Structural basis for competitive interactions of Pex14 with the import receptors Pex5 and Pex19."EMBO J. 28:745-754(2009) [PubMed] [Europe PMC] [Abstract]
  19. "Structural basis for docking of peroxisomal membrane protein carrier Pex19p onto its receptor Pex3p."EMBO J. 29:4083-4093(2010) [PubMed] [Europe PMC] [Abstract]
  20. "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]