PEX3

Peroxisomal biogenesis factor 3
Available structures PDB Ortholog search: PDBe, RCSB List of PDB id codes 3AJB​, 3MK4​
Identifiers
Symbols	PEX3 ; PBD10A; TRG18
External IDs	OMIM: 603164 MGI: 1929646 HomoloGene: 2691 GeneCards: PEX3 Gene
Gene ontology Molecular function • protein binding • lipid binding • amino acid transmembrane transporter activity • protein dimerization activity Cellular component • nucleoplasm • peroxisome • peroxisomal membrane • integral component of peroxisomal membrane • endoplasmic reticulum • cytosol • membrane • protein-lipid complex • intracellular membrane-bounded organelle • protein complex Biological process • positive regulation of defense response to virus by host • amino acid transmembrane transport • peroxisome organization • peroxisome membrane biogenesis • protein import into peroxisome membrane • transmembrane transport • activation of mitophagy in response to mitochondrial depolarization Sources: Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	8504	56535
Ensembl	ENSG00000034693	ENSMUSG00000019809
UniProt	P56589	Q9QXY9
RefSeq (mRNA)	NM_003630	NM_001164195
RefSeq (protein)	NP_003621	NP_001157667
Location (UCSC)	Chr 6: 143.45 – 143.49 Mb	Chr 10: 13.52 – 13.55 Mb
PubMed search

Peroxisomal biogenesis factor 3 is a protein that in humans is encoded by the PEX3 gene.^[1][2]

Interactions

PEX3 has been shown to interact with PEX19.^[3][4][5][6]

References

1. Kammerer S, Holzinger A, Welsch U, Roscher AA (Jun 1998). "Cloning and characterization of the gene encoding the human peroxisomal assembly protein Pex3p". FEBS Letters 429 (1): 53–60. doi:10.1016/S0014-5793(98)00557-2. PMID 9657383.
2. "Entrez Gene: PEX3 peroxisomal biogenesis factor 3".
3. Rual JF, Venkatesan K, Hao T, Hirozane-Kishikawa T, Dricot A, Li N, Berriz GF, Gibbons FD, Dreze M, Ayivi-Guedehoussou N, Klitgord N, Simon C, Boxem M, Milstein S, Rosenberg J, Goldberg DS, Zhang LV, Wong SL, Franklin G, Li S, Albala JS, Lim J, Fraughton C, Llamosas E, Cevik S, Bex C, Lamesch P, Sikorski RS, Vandenhaute J, Zoghbi HY, Smolyar A, Bosak S, Sequerra R, Doucette-Stamm L, Cusick ME, Hill DE, Roth FP, Vidal M (Oct 2005). "Towards a proteome-scale map of the human protein-protein interaction network". Nature 437 (7062): 1173–8. doi:10.1038/nature04209. PMID 16189514.
4. Mayerhofer PU, Kattenfeld T, Roscher AA, Muntau AC (Mar 2002). "Two splice variants of human PEX19 exhibit distinct functions in peroxisomal assembly". Biochemical and Biophysical Research Communications 291 (5): 1180–6. doi:10.1006/bbrc.2002.6568. PMID 11883941.
5. Sacksteder KA, Jones JM, South ST, Li X, Liu Y, Gould SJ (Mar 2000). "PEX19 binds multiple peroxisomal membrane proteins, is predominantly cytoplasmic, and is required for peroxisome membrane synthesis". The Journal of Cell Biology 148 (5): 931–44. doi:10.1083/jcb.148.5.931. PMC 2174547. PMID 10704444.
6. Fransen M, Wylin T, Brees C, Mannaerts GP, Van Veldhoven PP (Jul 2001). "Human pex19p binds peroxisomal integral membrane proteins at regions distinct from their sorting sequences". Molecular and Cellular Biology 21 (13): 4413–24. doi:10.1128/MCB.21.13.4413-4424.2001. PMC 87101. PMID 11390669.

Further reading

• South ST, Gould SJ (Jan 1999). "Peroxisome synthesis in the absence of preexisting peroxisomes". The Journal of Cell Biology 144 (2): 255–66. doi:10.1083/jcb.144.2.255. PMC 2132891. PMID 9922452.
• Soukupova M, Sprenger C, Gorgas K, Kunau WH, Dodt G (Jun 1999). "Identification and characterization of the human peroxin PEX3". European Journal of Cell Biology 78 (6): 357–74. doi:10.1016/S0171-9335(99)80078-8. PMID 10430017.
• Muntau AC, Holzinger A, Mayerhofer PU, Gärtner J, Roscher AA, Kammerer S (Feb 2000). "The human PEX3 gene encoding a peroxisomal assembly protein: genomic organization, positional mapping, and mutation analysis in candidate phenotypes". Biochemical and Biophysical Research Communications 268 (3): 704–10. doi:10.1006/bbrc.2000.2193. PMID 10679269.
• Sacksteder KA, Jones JM, South ST, Li X, Liu Y, Gould SJ (Mar 2000). "PEX19 binds multiple peroxisomal membrane proteins, is predominantly cytoplasmic, and is required for peroxisome membrane synthesis". The Journal of Cell Biology 148 (5): 931–44. doi:10.1083/jcb.148.5.931. PMC 2174547. PMID 10704444.
• Ghaedi K, Tamura S, Okumoto K, Matsuzono Y, Fujiki Y (Jun 2000). "The peroxin pex3p initiates membrane assembly in peroxisome biogenesis". Molecular Biology of the Cell 11 (6): 2085–102. doi:10.1091/mbc.11.6.2085. PMC 14905. PMID 10848631.
• Muntau AC, Mayerhofer PU, Paton BC, Kammerer S, Roscher AA (Oct 2000). "Defective peroxisome membrane synthesis due to mutations in human PEX3 causes Zellweger syndrome, complementation group G". American Journal of Human Genetics 67 (4): 967–75. doi:10.1086/303071. PMC 1287898. PMID 10958759.
• Ghaedi K, Honsho M, Shimozawa N, Suzuki Y, Kondo N, Fujiki Y (Oct 2000). "PEX3 is the causal gene responsible for peroxisome membrane assembly-defective Zellweger syndrome of complementation group G". American Journal of Human Genetics 67 (4): 976–81. doi:10.1086/303086. PMC 1287899. PMID 10968777.
• Fransen M, Wylin T, Brees C, Mannaerts GP, Van Veldhoven PP (Jul 2001). "Human pex19p binds peroxisomal integral membrane proteins at regions distinct from their sorting sequences". Molecular and Cellular Biology 21 (13): 4413–24. doi:10.1128/MCB.21.13.4413-4424.2001. PMC 87101. PMID 11390669.
• Mayerhofer PU, Kattenfeld T, Roscher AA, Muntau AC (Mar 2002). "Two splice variants of human PEX19 exhibit distinct functions in peroxisomal assembly". Biochemical and Biophysical Research Communications 291 (5): 1180–6. doi:10.1006/bbrc.2002.6568. PMID 11883941.
• Fransen M, Brees C, Ghys K, Amery L, Mannaerts GP, Ladant D, Van Veldhoven PP (Mar 2002). "Analysis of mammalian peroxin interactions using a non-transcription-based bacterial two-hybrid assay". Molecular & Cellular Proteomics 1 (3): 243–52. doi:10.1074/mcp.M100025-MCP200. PMID 12096124.
• Muntau AC, Roscher AA, Kunau WH, Dodt G (Jul 2003). "The interaction between human PEX3 and PEX19 characterized by fluorescence resonance energy transfer (FRET) analysis". European Journal of Cell Biology 82 (7): 333–42. doi:10.1078/0171-9335-00325. PMID 12924628.
• Muntau AC, Roscher AA, Kunau WH, Dodt G (2004). "Interaction of PEX3 and PEX19 visualized by fluorescence resonance energy transfer (FRET)". Advances in Experimental Medicine and Biology 544: 221–4. doi:10.1007/978-1-4419-9072-3_27. PMID 14713233.
• Fang Y, Morrell JC, Jones JM, Gould SJ (Mar 2004). "PEX3 functions as a PEX19 docking factor in the import of class I peroxisomal membrane proteins". The Journal of Cell Biology 164 (6): 863–75. doi:10.1083/jcb.200311131. PMC 2172291. PMID 15007061.
• Rual JF, Venkatesan K, Hao T, Hirozane-Kishikawa T, Dricot A, Li N, Berriz GF, Gibbons FD, Dreze M, Ayivi-Guedehoussou N, Klitgord N, Simon C, Boxem M, Milstein S, Rosenberg J, Goldberg DS, Zhang LV, Wong SL, Franklin G, Li S, Albala JS, Lim J, Fraughton C, Llamosas E, Cevik S, Bex C, Lamesch P, Sikorski RS, Vandenhaute J, Zoghbi HY, Smolyar A, Bosak S, Sequerra R, Doucette-Stamm L, Cusick ME, Hill DE, Roth FP, Vidal M (Oct 2005). "Towards a proteome-scale map of the human protein-protein interaction network". Nature 437 (7062): 1173–8. doi:10.1038/nature04209. PMID 16189514.
• Matsuzono Y, Matsuzaki T, Fujiki Y (Sep 2006). "Functional domain mapping of peroxin Pex19p: interaction with Pex3p is essential for function and translocation". Journal of Cell Science 119 (Pt 17): 3539–50. doi:10.1242/jcs.03100. PMID 16895967.

External links

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