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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
General
peroxisomal membrane protein (peroxin)
phosphoprotein
prenyl protein
Properties
SIZE: entity length = 299 aa
MW = 33 kD
COMPARTMENT: peroxisome
cytoplasm
MOTIF: essential {1-91}
MOTIF: binding site
SITE: 1-56
FOR-BINDING-OF: peroxin-3
Ser phosphorylation site {S35}
Ser phosphorylation site {S54}
Ser phosphorylation site {S146}
Ser phosphorylation site {S147}
Ser phosphorylation site {S149}
Ser phosphorylation site {S177}
Ser phosphorylation site {S184}
CaaX motif
SITE: C-TERMINUS
QUALITIES: AAX-TRUNCATED
CARBOXYMETHYLATED
EFFECTOR-BOUND: farnesyl
BOUND-VIA: cysteine residue
Database Correlations
OMIM correlations
UniProt P40855
Pfam PF04614
Entrez Gene 5824
Kegg hsa:5824
References
- UniProt :accession P40855
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=PEX19