| Standard Name | PEX13 1, 2 |
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| Systematic Name | YLR191W |
| Alias | PAS20 3 |
| Feature Type | ORF, Verified |
| Description | Integral peroxisomal membrane protein required for translocation of peroxisomal matrix proteins, interacts with the PTS1 signal recognition factor Pex5p and the PTS2 signal recognition factor Pex7p, forms a complex with Pex14p and Pex17p (4, 5, 6, 7 and see Summary Paragraph) |
| Name Description | PEroXin 2 |
| Chromosomal Location | |
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| View Computational GO annotations for PEX13 | |
| Molecular Function | |
| Manually curated | |
| Biological Process | |
| Manually curated | |
| Cellular Component | |
| Manually curated |
| Classical genetics | |
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| null | |
| reduction of function | |
| Large-scale survey | |
| null |
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| overexpression | |
| Resources |
| 185 total interaction(s) for 122 unique genes/features. | |
| Physical Interactions |
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| Genetic Interactions |
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| Resources |
| Localization | |
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| Phosphorylation | PhosphoGRID | PhosphoPep Database |
| Structure | |
| Homologs |
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| Last Update | Coordinates: 2011-02-03 | Sequence: 1996-07-31 | ||||||||||||
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| S288C only | |
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| S288C vs. other species | |
| S288C vs. other strains |
| External Links | All Associated Seq | Entrez Gene | Entrez RefSeq Protein | MIPS | Search all NCBI (Entrez) | UniProtKB |
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| Primary SGDID | S000004181 |
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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, 10, 11 and references therein).
Pex13p, Pex14p, and Pex17p form a docking complex that is required for PTS1 and PTS2 peroxisomal matrix proteins to associate with the peroxisomal membrane prior to import into the peroxisomal matrix (reviewed in 12, 13). Although Pex13p and Pex14p interact with both Pex5p (the receptor for PTS1) and Pex7p (the receptor for PTS2), Pex14p may be the initial docking site (14, 4, 15, 15). All members of the docking complex are localized to the cytosolic face of the peroxisomal membrane. Pex13 is an integral membrane protein with its amino and carboxy termini exposed to the cytoplasm, while Pex14 and Pex17 are peripheral membrane proteins (4, 14, 16). Therefore, interactions between the docking complex and the translocation complex comprised of Pex2p, Pex10p, and Pex12p may enable communication between both sides of the peroxisomal membrane (17).
Multiple interactions occur among the proteins of the docking complex. Pex14p interacts with Pex13p, an interaction that may be mediated by Pex5p (14, 18). Pex14p also interacts with Pex17p, even in the absence of Pex13p (16). Biochemical purification of the docking complex indicates that only 10% of Pex13p is located there, suggesting Pex13p may have additional functions (17, 19).
Of the three proteins, Pex13p and Pex14 are widely conserved but Pex17p has only been identified in ascomycetes such as S. cerevisiae and C. albicans (20). Mutations in the human Pex13 and Pex14 have been associated with the peroxisomal biogenesis disorders neonatal adrenoleukodystrophy and Zellweger syndrome (21, 22, and references therein).
| 1) | Erdmann R and Blobel G (1996) Identification of Pex13p a peroxisomal membrane receptor for the PTS1 recognition factor. J Cell Biol 135(1):111-21 |
| 2) | Distel B, et al. (1996) A unified nomenclature for peroxisome biogenesis factors. J Cell Biol 135(1):1-3 |
| 3) | Elgersma Y, et al. (1993) An efficient positive selection procedure for the isolation of peroxisomal import and peroxisome assembly mutants of Saccharomyces cerevisiae. Genetics 135(3):731-40 |
| 4) | Girzalsky W, et al. (1999) Involvement of Pex13p in Pex14p localization and peroxisomal targeting signal 2-dependent protein import into peroxisomes. J Cell Biol 144(6):1151-62 |
| 5) | Gould SJ, et al. (1996) Pex13p is an SH3 protein of the peroxisome membrane and a docking factor for the predominantly cytoplasmic PTs1 receptor. J Cell Biol 135(1):85-95 |
| 6) | Pires JR, et al. (2003) The ScPex13p SH3 domain exposes two distinct binding sites for Pex5p and Pex14p. J Mol Biol 326(5):1427-35 |
| 7) | Girzalsky W, et al. (2006) Pex19p-dependent targeting of Pex17p, a peripheral component of the peroxisomal protein import machinery. J Biol Chem 281(28):19417-25 |
| 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) | Purdue PE and Lazarow PB (2001) Peroxisome biogenesis. Annu Rev Cell Dev Biol 17:701-52 |
| 11) | 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 |
| 12) | Heiland I and Erdmann R (2005) Biogenesis of peroxisomes. Topogenesis of the peroxisomal membrane and matrix proteins. FEBS J 272(10):2362-72 |
| 13) | Brown LA and Baker A (2003) Peroxisome biogenesis and the role of protein import. J Cell Mol Med 7(4):388-400 |
| 14) | Albertini M, et al. (1997) Pex14p, a peroxisomal membrane protein binding both receptors of the two PTS-dependent import pathways. Cell 89(1):83-92 |
| 15) | Niederhoff K, et al. (2005) Yeast Pex14p possesses two functionally distinct Pex5p and one Pex7p binding sites. J Biol Chem 280(42):35571-8 |
| 16) | Huhse B, et al. (1998) Pex17p of Saccharomyces cerevisiae is a novel peroxin and component of the peroxisomal protein translocation machinery. J Cell Biol 140(1):49-60 |
| 17) | Agne B, et al. (2003) Pex8p: an intraperoxisomal organizer of the peroxisomal import machinery. Mol Cell 11(3):635-46 |
| 18) | Schell-Steven A, et al. (2005) Identification of a novel, intraperoxisomal pex14-binding site in pex13: association of pex13 with the docking complex is essential for peroxisomal matrix protein import. Mol Cell Biol 25(8):3007-18 |
| 19) | Williams C and Distel B (2006) Pex13p: Docking or cargo handling protein? Biochim Biophys Acta 1763(12):1585-91 |
| 20) | Kiel JA, et al. (2006) PEX Genes in Fungal Genomes: Common, Rare or Redundant. Traffic 7(10):1291-303 |
| 21) | Bottger G, et al. (2000) Saccharomyces cerevisiae PTS1 receptor Pex5p interacts with the SH3 domain of the peroxisomal membrane protein Pex13p in an unconventional, non-PXXP-related manner. Mol Biol Cell 11(11):3963-76 |
| 22) | Azevedo JE and Schliebs W (2006) Pex14p, more than just a docking protein. Biochim Biophys Acta 1763(12):1574-84 |





