PEX10/YDR265W Summary Help

Standard Name PEX10 1
Systematic Name YDR265W
Alias PAS4
Feature Type ORF, Verified
Description Peroxisomal membrane E3 ubiquitin ligase; required for for Ubc4p-dependent Pex5p ubiquitination and peroxisomal matrix protein import; contains zinc-binding RING domain; mutations in human homolog cause various peroxisomal disorders (2, 3, 4, 5, 6, 7 and see Summary Paragraph)
Name Description PEroXin 1
Chromosomal Location
ChrIV:998864 to 999877 | ORF Map | GBrowse
Gene Ontology Annotations All PEX10 GO evidence and references
  View Computational GO annotations for PEX10
Molecular Function
Manually curated
Biological Process
Manually curated
Cellular Component
Manually curated
Regulators 3 genes
Classical genetics
reduction of function
Large-scale survey
89 total interaction(s) for 63 unique genes/features.
Physical Interactions
  • Affinity Capture-MS: 4
  • Affinity Capture-RNA: 1
  • Affinity Capture-Western: 15
  • Biochemical Activity: 3
  • Co-fractionation: 3
  • Co-purification: 1
  • PCA: 13

Genetic Interactions
  • Dosage Growth Defect: 1
  • Dosage Lethality: 1
  • Negative Genetic: 27
  • Phenotypic Enhancement: 1
  • Positive Genetic: 10
  • Synthetic Growth Defect: 7
  • Synthetic Lethality: 2

Expression Summary
Length (a.a.) 337
Molecular Weight (Da) 39,099
Isoelectric Point (pI) 9.88
Phosphorylation PhosphoGRID | PhosphoPep Database
sequence information
ChrIV:998864 to 999877 | ORF Map | GBrowse
Last Update Coordinates: 2011-02-03 | Sequence: 1996-07-31
Subfeature details
Most Recent Updates
Coordinates Sequence
CDS 1..1014 998864..999877 2011-02-03 1996-07-31
Retrieve sequences
Analyze Sequence
S288C only
S288C vs. other species
S288C vs. other strains
External Links All Associated Seq | Entrez Gene | Entrez RefSeq Protein | MIPS | Search all NCBI (Entrez) | UniProtKB
Primary SGDIDS000002673

The biogenesis of peroxisomes requires a group of protein factors referred to as peroxins which are encoded by the PEX genes. Peroxisomal proteins are synthesized on free polyribosomes and imported posttranslationally. The transport of peroxisomal matrix proteins from the cytoplasm to the peroxisome is mediated by two peroxisome-targeting signal sequences (PTS1 and PTS2). Peroxisomal membrane proteins (PMPs) are imported independently of the matrix proteins by a distinct mechanism mediated by the membrane PTS signal (mPTS) (8, 9, 4, 10 and references therein).

Pex10p is a peroxisomal membrane protein required for peroxisome biogenesis and peroxisomal matrix protein import (5, 11). It exhibits E3 ubiquitin ligase activity in vitro, and is required for Ubc4p-dependent ubiquitination of Pex5p, but not for Pex4p-dependent ubiquitination of Pex5p (6).

Two subcomplexes of the peroxisomal import machinery have been defined: the docking subcomplex comprises Pex14p, Pex17p, and Pex13p, while the translocation subcomplex contains Pex2p, Pex10p, and Pex12p. The proteins of the translocation complex expose their RING finger domains to the outer face of the peroxisomal membrane, and act downstream of Pex14p, Pex17p, and Pex13p during the peroxisomal protein import process (12). Association of both subcomplexes into a larger import complex requires Pex8p, an intraperoxisomal protein. Pex8p organizes the formation of the larger import complex from the trans side of the peroxisomal membrane and thus might enable functional communication between both sides of the membrane (12).

Pex10p also plays a central role in the peroxisomal protein interaction network by connecting the ubiquitin conjugating enzyme Pex4p to the other members of the peroxisomal protein import machinery, and is required for the ubiquitination of the PTS1 (peroxisomal targeting signal 1) receptor Pex5p (5, 12, 13, 14). pex10 null mutants are viable but peroxisome deficient, mislocalize peroxisomal matrix proteins to the cytosol, and accumulate Pex5p at the cytosolic face of the peroxisomal membrane (5, 11).

The human peroxisome biogenesis disorders (PBDs) are a group of genetically heterogeneous diseases characterized by severe mental retardation, neuronal, hepatic and renal abnormalities, and death in early infancy (2). Clinical features of PBD patients vary, but all exhibit a defect in the import of one or more classes of peroxisomal matrix proteins. This cellular phenotype is shared by yeast pex mutants, and human orthologs of yeast PEX genes are defective in some groups of PBD patients (2, 15).

Mutations in human PEX10 have been associated with Zellweger Syndrome and adrenoleukodystrophy.

Last updated: 2007-06-26 Contact SGD

References cited on this page View Complete Literature Guide for PEX10
1) Distel B, et al.  (1996) A unified nomenclature for peroxisome biogenesis factors. J Cell Biol 135(1):1-3
2) Warren DS, et al.  (1998) Identification of PEX10, the gene defective in complementation group 7 of the peroxisome-biogenesis disorders. Am J Hum Genet 63(2):347-59
3) Albertini M, et al.  (2001) Pex12p of Saccharomyces cerevisiae is a component of a multi-protein complex essential for peroxisomal matrix protein import. Eur J Cell Biol 80(4):257-70
4) Purdue PE and Lazarow PB  (2001) Peroxisome biogenesis. Annu Rev Cell Dev Biol 17:701-52
5) Eckert JH and Johnsson N  (2003) Pex10p links the ubiquitin conjugating enzyme Pex4p to the protein import machinery of the peroxisome. J Cell Sci 116(Pt 17):3623-34
6) Williams C, et al.  (2008) Pex10p functions as an E3 ligase for the Ubc4p-dependent ubiquitination of Pex5p. Biochem Biophys Res Commun 374(4):620-4
7) Platta HW, et al.  (2009) Pex2 and pex12 function as protein-ubiquitin ligases in peroxisomal protein import. Mol Cell Biol 29(20):5505-16
8) Hettema EH, et al.  (2000) Saccharomyces cerevisiae pex3p and pex19p are required for proper localization and stability of peroxisomal membrane proteins. EMBO J 19(2):223-33
9) Sacksteder KA and Gould SJ  (2000) The genetics of peroxisome biogenesis. Annu Rev Genet 34:623-652
10) Fujiki Y, et al.  (2006) Import of peroxisomal membrane proteins: The interplay of Pex3p- and Pex19p-mediated interactions. Biochim Biophys Acta 1763(12):1639-46
11) Van der Leij I, et al.  (1992) Isolation of peroxisome assembly mutants from Saccharomyces cerevisiae with different morphologies using a novel positive selection procedure. J Cell Biol 119(1):153-62
12) Agne B, et al.  (2003) Pex8p: an intraperoxisomal organizer of the peroxisomal import machinery. Mol Cell 11(3):635-46
13) Kiel JA, et al.  (2005) Ubiquitination of the peroxisomal targeting signal type 1 receptor, Pex5p, suggests the presence of a quality control mechanism during peroxisomal matrix protein import. J Biol Chem 280(3):1921-30
14) Platta HW, et al.  (2004) Ubiquitination of the peroxisomal import receptor Pex5p. Biochem J 384(Pt 1):37-45
15) Chang CC, et al.  (1997) Isolation of the human PEX12 gene, mutated in group 3 of the peroxisome biogenesis disorders. Nat Genet 15(4):385-8