Peroxisomal Biogenesis Factor 13 (PEX13)

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NALD; ZWS; Peroxin-13; Peroxisomal membrane protein PEX13

Peroxisomal Biogenesis Factor 13 (PEX13)
PEX13 functions as a docking factor for the predominantly cytoplasmic PTS1 receptor. PTS1 consists of a C-terminal tripeptide with the sequence ser-lys-leu or a conservative variant and is used by almost all proteins destined for the peroxisomal lumen. PTS2, also directs proteins to the peroxisomal lumen but differs from PTS1 because it is found at the N terminus and is used much less commonly. Integral peroxisomal membrane proteins use neither PTS1 nor PTS2, but rather a distinct type of signal. Pex13p SH3 interacted specifically with Pex5p in vitro. Pex5p was present mainly in the cytosol and only a small fraction was associated with peroxisomes. Pex13p is a component of the peroxisomal protein import machinery onto which the mobile pex5p receptor docks for the delivery of the selected PTS1 protein.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Peroxisomal Biogenesis Factor 13 (PEX13) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Peroxisomal Biogenesis Factor 13 (PEX13) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Peroxisomal Biogenesis Factor 13 (PEX13) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Peroxisomal Biogenesis Factor 13 (PEX13) CLIA Kit Customized Service Offer
n/a ELISA Kit for Peroxisomal Biogenesis Factor 13 (PEX13) ELISA Kit Customized Service Offer

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Peroxisomal Biogenesis Factor 13 (PEX13) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Peroxisomal Biogenesis Factor 13 (PEX13) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Peroxisomal Biogenesis Factor 13 (PEX13) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Peroxisomal Biogenesis Factor 13 (PEX13) CLIA Kit Customized Service Offer
n/a ELISA Kit for Peroxisomal Biogenesis Factor 13 (PEX13) ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Peroxisomal Biogenesis Factor 13 (PEX13) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Peroxisomal Biogenesis Factor 13 (PEX13) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Peroxisomal Biogenesis Factor 13 (PEX13) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Peroxisomal Biogenesis Factor 13 (PEX13) CLIA Kit Customized Service Offer
n/a ELISA Kit for Peroxisomal Biogenesis Factor 13 (PEX13) ELISA Kit Customized Service Offer
  1. "Identification of a human PTS1 receptor docking protein directly required for peroxisomal protein import."Proc. Natl. Acad. Sci. U.S.A. 95:8087-8092(1998) [PubMed] [Europe PMC] [Abstract]
  2. "Isolation, characterization and mutation analysis of PEX13-defective Chinese hamster ovary cell mutants."Hum. Mol. Genet. 8:1673-1681(1999) [PubMed] [Europe PMC] [Abstract]
  3. "Complete sequencing and characterization of 21,243 full-length human cDNAs." Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
  4. "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]
  5. "Pex13p is an SH3 protein of the peroxisome membrane and a docking factor for the predominantly cytoplasmic PTs1 receptor."J. Cell Biol. 135:85-95(1996) [PubMed] [Europe PMC] [Abstract]
  6. "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]
  7. "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]
  8. "Zellweger syndrome caused by PEX13 deficiency: report of two novel mutations."Am. J. Med. Genet. A 149:1219-1223(2009) [PubMed] [Europe PMC] [Abstract]
  9. "PEX13 is mutated in complementation group 13 of the peroxisome-biogenesis disorders."Am. J. Hum. Genet. 65:621-634(1999) [PubMed] [Europe PMC] [Abstract]
  10. "Nonsense and temperature-sensitive mutations in PEX13 are the cause of complementation group H of peroxisome biogenesis disorders."Hum. Mol. Genet. 8:1077-1083(1999) [PubMed] [Europe PMC] [Abstract]