PEX5

PEX5
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	1FCH, 2C0L, 2C0M, 2J9Q, 2W84, 3R9A, 4BXU, 4KXK, 4KYO
Identifiers
Aliases	PEX5, peroxisomal biogenesis factor 5, RCDP5, PTS1R, PBD2B, PTS1-BP, PXR1, PBD2A
External IDs	OMIM: 600414; MGI: 1098808; HomoloGene: 270; GeneCards: PEX5; OMA:PEX5 - orthologs
Gene location (Human)
Chr.	Chromosome 12 (human)
End	7,218,574 bp
Gene location (Mouse)
Chr.	Chromosome 6 (mouse)
End	124,392,026 bp
RNA expression pattern
	Top expressed in
	gastrocnemius muscle; ; left testis; ; right testis; ; C1 segment; ; skeletal muscle tissue; ; substantia nigra; ; prefrontal cortex; ; putamen; ; hippocampus proper; ; right frontal lobe;
	Top expressed in
	right kidney; ; left lobe of liver; ; granulocyte; ; proximal tubule; ; lip; ; spermatocyte; ; muscle of thigh; ; ventricular zone; ; stroma of bone marrow; ; brown adipose tissue;
	More reference expression data
	; ;
	More reference expression data
Gene ontology
Molecular function	protein C-terminus binding; small GTPase binding; protein N-terminus binding; peroxisome targeting sequence binding; enzyme binding; protein binding; peroxisome matrix targeting signal-1 binding;
Cellular component	cytoplasm; peroxisome; membrane; peroxisomal matrix; intracellular anatomical structure; Golgi apparatus; cytosol; peroxisomal membrane; protein-containing complex;
Biological process	negative regulation of protein homotetramerization; protein import into peroxisome membrane; protein import into peroxisome matrix, docking; protein tetramerization; protein transport; protein targeting to peroxisome; protein import into peroxisome matrix, translocation; protein import into peroxisome matrix; protein ubiquitination; transport;
	Sources:Amigo / QuickGO
Orthologs
	5830
	19305
	ENSG00000139197; ENSG00000288217
	ENSMUSG00000005069
	P50542
	O09012
NM_000319; NM_001131023; NM_001131024; NM_001131025; NM_001131026;
	NM_001300789; NM_001351124; NM_001351126; NM_001351127; NM_001351128; NM_001351130; NM_001351131; NM_001351132; NM_001351133; NM_001351134; NM_001351135; NM_001351136; NM_001351137; NM_001351138; NM_001351139; NM_001351140; NM_001374645; NM_001374646; NM_001374647; NM_001374648; NM_001374649
	NM_001277330; NM_001277805; NM_008995; NM_175933; NM_001360570
NP_000310; NP_001124495; NP_001124496; NP_001124497; NP_001124498;
	NP_001287718; NP_001338053; NP_001338055; NP_001338056; NP_001338057; NP_001338059; NP_001338060; NP_001338061; NP_001338062; NP_001338063; NP_001338064; NP_001338065; NP_001338066; NP_001338067; NP_001338068; NP_001338069
	NP_001264259; NP_001264734; NP_033021; NP_787947; NP_001347499
	Wikidata
View/Edit Human	View/Edit Mouse

Peroxisomal targeting signal 1 receptor (PTS1R) is a protein that in humans is encoded by the PEX5 gene.

PTS1R is a peroxisomal targeting sequence involved in the specific transport of molecules for oxidation inside the peroxisome. SKL binds to PTS1R in the cytosol followed by binding to the Pex14p receptor allowing importation of the peroxisomal protein through the pexsubunit transporter.

Diseases associated with dysfunctional PTS1R receptors include X-linked adrenoleukodystrophy and Zellweger syndrome.