Family

Peroxin-3 (IPR006966)

Short name: Peroxin-3

Overlapping homologous superfamilies

None.

Family relationships

None.

Description

Peroxin 3 (Pex3p), also known as Peroxisomal biogenesis factor 3, has been identified and characterised as a peroxisomal membrane protein in yeasts and mammals [PMID: 14733948]. Two putative peroxisomal membrane-bound Pex3p homologues have also been found in Arabidopsis thaliana [PMID: 14733948]. They possess a membrane peroxisomal targeting signal. Pex3p is an integral membrane protein of peroxisomes, exposing its N- and C-terminal parts to the cytosol [PMID: 10848631]. Peroxin is involved in peroxisome biosynthesis and integrity; it assembles membrane vesicles before the matrix proteins are translocated.

In humans, defects in PEX3 are the cause of peroxisome biogenesis disorders [PMID: 10968777], which include Zellweger syndrome (ZWS), neonatal adrenoleukodystrophy (NALD), infantile Refsum disease (IRD), and classical rhizomelic chondrodysplasia punctata (RCDP). These are peroxisomal disorders that are the result of proteins failing to be imported into the peroxisome.

GO terms

Biological Process

GO:0007031 peroxisome organization

Molecular Function

No terms assigned in this category.

Cellular Component

GO:0005779 integral component of peroxisomal membrane

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.
Pfam
PANTHER