peroxisomal biogenesis factor 19; peroxin-19; peroxisomal farnesylated protein; 33 kD housekeeping protein (PEX19, HK33, PXF, OK/SW-cl22)

Function

• necessary for early peroxisomal biogenesis
• acts both as a cytosolic chaperone & as an import receptor for peroxisomal membrane proteins
• binds & stabilizes newly synthesized peroxisomal membrane proteins in the cytoplasm by interacting with their hydrophobic membrane-spanning domains, & targets them to the peroxisome membrane by binding to the integral membrane protein PEX3
• excludes CDKN2A from the nucleus & prevents its interaction with MDM2, which results in active degradation of TP53
• interacts with a broad range of peroxisomal membrane proteins, including PEX3, PEX10, PEX11A, PEX11B, PEX12, PEX13, PEX14 & PEX16, PXMP2/PMP22, PXMP4/PMP24, SLC25A17/PMP34, ABCD1/ALDP, ABCD2/ALDRP, & ABCD3/PMP70
• also interacts with the tumor suppressor CDKN2A/p19ARF

Structure

belongs to the peroxin-19 family

Compartment

• cytoplasm
• peroxisome membrane
• lipid-anchor; cytoplasmic side
• mainly cytoplasmic
• some fraction membrane-associated to the outer surface of peroxisomes

Alternative splicing

named isoforms=5

Expression

• ubiquitously expressed
• isoform 1 is strongly predominant in all tissues except in utero where isoform 2 is the main form

Pathology

• defects in PEX19 are the cause of peroxisome biogenesis disorder complementation group 14
• defects in PEX19 are a cause of Zellweger syndrome

More general terms

• peroxisomal membrane protein (peroxin)
• prenyl protein
• phosphoprotein

Database

• UniProt: http://www.uniprot.org/uniprot/P40855.html
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=5824
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:5824
• OMIM: https://mirror.omim.org/entry/214100
• OMIM: https://mirror.omim.org/entry/600279
• OMIM: https://mirror.omim.org/entry/601539