PEX5

Peroxisomal biogenesis factor 5
PDB rendering based on 1fch.
Available structures PDB Ortholog search: PDBe, RCSB List of PDB id codes 1FCH​, 2C0L​, 2C0M​, 2J9Q​, 2W84​, 3R9A​, 4BXU​, 4KXK​, 4KYO​
Identifiers
Symbols	PEX5 ; PBD2A; PBD2B; PTS1-BP; PTS1R; PXR1
External IDs	OMIM: 600414 MGI: 1098808 HomoloGene: 270 GeneCards: PEX5 Gene
Gene ontology Molecular function • peroxisome targeting sequence binding • peroxisome matrix targeting signal-1 binding • protein binding • protein C-terminus binding • enzyme binding • small GTPase binding • protein N-terminus binding Cellular component • intracellular • cytoplasm • peroxisome • peroxisomal membrane • peroxisomal matrix • Golgi apparatus • cytosol • membrane • protein complex Biological process • protein targeting to peroxisome • protein import into peroxisome matrix • protein import into peroxisome matrix, docking • protein import into peroxisome matrix, translocation • protein import into peroxisome membrane • protein tetramerization • negative regulation of protein homotetramerization Sources: Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	5830	19305
Ensembl	ENSG00000139197	ENSMUSG00000005069
UniProt	P50542	O09012
RefSeq (mRNA)	NM_000319	NM_001277330
RefSeq (protein)	NP_000310	NP_001264259
Location (UCSC)	Chr 12: 7.19 – 7.22 Mb	Chr 6: 124.4 – 124.42 Mb
PubMed search

Peroxisomal targeting signal 1 receptor is a protein that in humans is encoded by the PEX5 gene.^[1]

Interactions

PEX5 has been shown to interact with PEX12,^[2][3] PEX13^[4][5] and PEX14.^[3][5][6]

References

1. "Entrez Gene: PEX5 Peroxisomal biogenesis factor 5".
2. Chang CC, Warren DS, Sacksteder KA, Gould SJ (Nov 1999). "PEX12 interacts with PEX5 and PEX10 and acts downstream of receptor docking in peroxisomal matrix protein import". The Journal of Cell Biology 147 (4): 761–74. doi:10.1083/jcb.147.4.761. PMC 2156163. PMID 10562279.
3. Okumoto K, Abe I, Fujiki Y (Aug 2000). "Molecular anatomy of the peroxin Pex12p: ring finger domain is essential for Pex12p function and interacts with the peroxisome-targeting signal type 1-receptor Pex5p and a ring peroxin, Pex10p". The Journal of Biological Chemistry 275 (33): 25700–10. doi:10.1074/jbc.M003303200. PMID 10837480.
4. Gould SJ, Kalish JE, Morrell JC, Bjorkman J, Urquhart AJ, Crane DI (Oct 1996). "Pex13p is an SH3 protein of the peroxisome membrane and a docking factor for the predominantly cytoplasmic PTs1 receptor". The Journal of Cell Biology 135 (1): 85–95. doi:10.1083/jcb.135.1.85. PMC 2121023. PMID 8858165.
5. Otera H, Setoguchi K, Hamasaki M, Kumashiro T, Shimizu N, Fujiki Y (Mar 2002). "Peroxisomal targeting signal receptor Pex5p interacts with cargoes and import machinery components in a spatiotemporally differentiated manner: conserved Pex5p WXXXF/Y motifs are critical for matrix protein import". Molecular and Cellular Biology 22 (6): 1639–55. doi:10.1128/MCB.22.6.1639-1655.2002. PMC 135613. PMID 11865044.
6. Shimizu N, Itoh R, Hirono Y, Otera H, Ghaedi K, Tateishi K, Tamura S, Okumoto K, Harano T, Mukai S, Fujiki Y (Apr 1999). "The peroxin Pex14p. cDNA cloning by functional complementation on a Chinese hamster ovary cell mutant, characterization, and functional analysis". The Journal of Biological Chemistry 274 (18): 12593–604. doi:10.1074/jbc.274.18.12593. PMID 10212238.

Further reading

• Fransen M, Brees C, Baumgart E, Vanhooren JC, Baes M, Mannaerts GP, Van Veldhoven PP (Mar 1995). "Identification and characterization of the putative human peroxisomal C-terminal targeting signal import receptor". The Journal of Biological Chemistry 270 (13): 7731–6. doi:10.1074/jbc.270.13.7731. PMID 7706321.
• Dodt G, Braverman N, Wong C, Moser A, Moser HW, Watkins P, Valle D, Gould SJ (Feb 1995). "Mutations in the PTS1 receptor gene, PXR1, define complementation group 2 of the peroxisome biogenesis disorders". Nature Genetics 9 (2): 115–25. doi:10.1038/ng0295-115. PMID 7719337.
• Wiemer EA, Nuttley WM, Bertolaet BL, Li X, Francke U, Wheelock MJ, Anné UK, Johnson KR, Subramani S (Jul 1995). "Human peroxisomal targeting signal-1 receptor restores peroxisomal protein import in cells from patients with fatal peroxisomal disorders". The Journal of Cell Biology 130 (1): 51–65. doi:10.1083/jcb.130.1.51. PMC 2120514. PMID 7790377.
• Maruyama K, Sugano S (Jan 1994). "Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides". Gene 138 (1-2): 171–4. doi:10.1016/0378-1119(94)90802-8. PMID 8125298.
• Marynen P, Fransen M, Raeymaekers P, Mannaerts GP, Van Veldhoven PP (Nov 1995). "The gene for the peroxisomal targeting signal import receptor (PXR1) is located on human chromosome 12p13, flanked by TPI1 and D12S1089". Genomics 30 (2): 366–8. doi:10.1006/geno.1995.0032. PMID 8586442.
• Gould SJ, Kalish JE, Morrell JC, Bjorkman J, Urquhart AJ, Crane DI (Oct 1996). "Pex13p is an SH3 protein of the peroxisome membrane and a docking factor for the predominantly cytoplasmic PTs1 receptor". The Journal of Cell Biology 135 (1): 85–95. doi:10.1083/jcb.135.1.85. PMC 2121023. PMID 8858165.
• Dodt G, Gould SJ (Dec 1996). "Multiple PEX genes are required for proper subcellular distribution and stability of Pex5p, the PTS1 receptor: evidence that PTS1 protein import is mediated by a cycling receptor". The Journal of Cell Biology 135 (6 Pt 2): 1763–74. doi:10.1083/jcb.135.6.1763. PMC 2133940. PMID 8991089.
• Suzuki Y, Yoshitomo-Nakagawa K, Maruyama K, Suyama A, Sugano S (Oct 1997). "Construction and characterization of a full length-enriched and a 5'-end-enriched cDNA library". Gene 200 (1-2): 149–56. doi:10.1016/S0378-1119(97)00411-3. PMID 9373149.
• Reuber BE, Germain-Lee E, Collins CS, Morrell JC, Ameritunga R, Moser HW, Valle D, Gould SJ (Dec 1997). "Mutations in PEX1 are the most common cause of peroxisome biogenesis disorders". Nature Genetics 17 (4): 445–8. doi:10.1038/ng1297-445. PMID 9398847.
• Otera H, Okumoto K, Tateishi K, Ikoma Y, Matsuda E, Nishimura M, Tsukamoto T, Osumi T, Ohashi K, Higuchi O, Fujiki Y (Jan 1998). "Peroxisome targeting signal type 1 (PTS1) receptor is involved in import of both PTS1 and PTS2: studies with PEX5-defective CHO cell mutants". Molecular and Cellular Biology 18 (1): 388–99. doi:10.1128/mcb.18.1.388. PMC 121509. PMID 9418886.
• Fransen M, Terlecky SR, Subramani S (Jul 1998). "Identification of a human PTS1 receptor docking protein directly required for peroxisomal protein import". Proceedings of the National Academy of Sciences of the United States of America 95 (14): 8087–92. doi:10.1073/pnas.95.14.8087. PMC 20933. PMID 9653144.
• Amery L, Brees C, Baes M, Setoyama C, Miura R, Mannaerts GP, Van Veldhoven PP (Dec 1998). "C-terminal tripeptide Ser-Asn-Leu (SNL) of human D-aspartate oxidase is a functional peroxisome-targeting signal". The Biochemical Journal 336 (Pt 2): 367–71. doi:10.1042/bj3360367. PMC 1219880. PMID 9820813.
• Shimizu N, Itoh R, Hirono Y, Otera H, Ghaedi K, Tateishi K, Tamura S, Okumoto K, Harano T, Mukai S, Fujiki Y (Apr 1999). "The peroxin Pex14p. cDNA cloning by functional complementation on a Chinese hamster ovary cell mutant, characterization, and functional analysis". The Journal of Biological Chemistry 274 (18): 12593–604. doi:10.1074/jbc.274.18.12593. PMID 10212238.
• Shimozawa N, Zhang Z, Suzuki Y, Imamura A, Tsukamoto T, Osumi T, Fujiki Y, Orii T, Barth PG, Wanders RJ, Kondo N (Aug 1999). "Functional heterogeneity of C-terminal peroxisome targeting signal 1 in PEX5-defective patients". Biochemical and Biophysical Research Communications 262 (2): 504–8. doi:10.1006/bbrc.1999.1232. PMID 10462504.
• Chang CC, Warren DS, Sacksteder KA, Gould SJ (Nov 1999). "PEX12 interacts with PEX5 and PEX10 and acts downstream of receptor docking in peroxisomal matrix protein import". The Journal of Cell Biology 147 (4): 761–74. doi:10.1083/jcb.147.4.761. PMC 2156163. PMID 10562279.
• Watters D, Kedar P, Spring K, Bjorkman J, Chen P, Gatei M, Birrell G, Garrone B, Srinivasa P, Crane DI, Lavin MF (Nov 1999). "Localization of a portion of extranuclear ATM to peroxisomes". The Journal of Biological Chemistry 274 (48): 34277–82. doi:10.1074/jbc.274.48.34277. PMID 10567403.
• Okumoto K, Abe I, Fujiki Y (Aug 2000). "Molecular anatomy of the peroxin Pex12p: ring finger domain is essential for Pex12p function and interacts with the peroxisome-targeting signal type 1-receptor Pex5p and a ring peroxin, Pex10p". The Journal of Biological Chemistry 275 (33): 25700–10. doi:10.1074/jbc.M003303200. PMID 10837480.
• Gatto GJ, Geisbrecht BV, Gould SJ, Berg JM (Dec 2000). "Peroxisomal targeting signal-1 recognition by the TPR domains of human PEX5". Nature Structural Biology 7 (12): 1091–5. doi:10.1038/81930. PMID 11101887.
• Harano T, Nose S, Uezu R, Shimizu N, Fujiki Y (Jul 2001). "Hsp70 regulates the interaction between the peroxisome targeting signal type 1 (PTS1)-receptor Pex5p and PTS1". The Biochemical Journal 357 (Pt 1): 157–65. doi:10.1042/0264-6021:3570157. PMC 1221938. PMID 11415446.
• Saidowsky J, Dodt G, Kirchberg K, Wegner A, Nastainczyk W, Kunau WH, Schliebs W (Sep 2001). "The di-aromatic pentapeptide repeats of the human peroxisome import receptor PEX5 are separate high affinity binding sites for the peroxisomal membrane protein PEX14". The Journal of Biological Chemistry 276 (37): 34524–9. doi:10.1074/jbc.M104647200. PMID 11438541.
• Stanley WA, Filipp FV, Kursula P, Schüller N, Erdmann R, Schliebs W, Sattler M, Wilmanns M (Dec 2006). "Recognition of a functional peroxisome type 1 target by the dynamic import receptor pex5p". Molecular Cell 24 (5): 653–63. doi:10.1016/j.molcel.2006.10.024. PMID 17157249.

External links

• GeneReviews/NCBI/NIH/UW entry on Peroxisome Biogenesis Disorders, Zellweger Syndrome Spectrum
• OMIM entries on Peroxisome Biogenesis Disorders, Zellweger Syndrome Spectrum