PEX3 explained

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]}

Further reading

• South ST, Gould SJ . Peroxisome synthesis in the absence of preexisting peroxisomes . The Journal of Cell Biology . 144 . 2 . 255–66 . Jan 1999 . 9922452 . 2132891 . 10.1083/jcb.144.2.255 .
• Soukupova M, Sprenger C, Gorgas K, Kunau WH, Dodt G . Identification and characterization of the human peroxin PEX3 . European Journal of Cell Biology . 78 . 6 . 357–74 . Jun 1999 . 10430017 . 10.1016/S0171-9335(99)80078-8 .
• Muntau AC, Holzinger A, Mayerhofer PU, Gärtner J, Roscher AA, Kammerer S . 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 . Feb 2000 . 10679269 . 10.1006/bbrc.2000.2193 .
• Sacksteder KA, Jones JM, South ST, Li X, Liu Y, Gould SJ . 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 . Mar 2000 . 10704444 . 2174547 . 10.1083/jcb.148.5.931 .
• Ghaedi K, Tamura S, Okumoto K, Matsuzono Y, Fujiki Y . The peroxin pex3p initiates membrane assembly in peroxisome biogenesis . Molecular Biology of the Cell . 11 . 6 . 2085–102 . Jun 2000 . 10848631 . 14905 . 10.1091/mbc.11.6.2085 .
• Muntau AC, Mayerhofer PU, Paton BC, Kammerer S, Roscher AA . 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 . Oct 2000 . 10958759 . 1287898 . 10.1086/303071 .
• Ghaedi K, Honsho M, Shimozawa N, Suzuki Y, Kondo N, Fujiki Y . 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 . Oct 2000 . 10968777 . 1287899 . 10.1086/303086 .
• Fransen M, Wylin T, Brees C, Mannaerts GP, Van Veldhoven PP . Human pex19p binds peroxisomal integral membrane proteins at regions distinct from their sorting sequences . Molecular and Cellular Biology . 21 . 13 . 4413–24 . Jul 2001 . 11390669 . 87101 . 10.1128/MCB.21.13.4413-4424.2001 .
• Mayerhofer PU, Kattenfeld T, Roscher AA, Muntau AC . Two splice variants of human PEX19 exhibit distinct functions in peroxisomal assembly . Biochemical and Biophysical Research Communications . 291 . 5 . 1180–6 . Mar 2002 . 11883941 . 10.1006/bbrc.2002.6568 .
• Fransen M, Brees C, Ghys K, Amery L, Mannaerts GP, Ladant D, Van Veldhoven PP . Analysis of mammalian peroxin interactions using a non-transcription-based bacterial two-hybrid assay . Molecular & Cellular Proteomics . 1 . 3 . 243–52 . Mar 2002 . 12096124 . 10.1074/mcp.M100025-MCP200 . free .
• Muntau AC, Roscher AA, Kunau WH, Dodt G . The interaction between human PEX3 and PEX19 characterized by fluorescence resonance energy transfer (FRET) analysis . European Journal of Cell Biology . 82 . 7 . 333–42 . Jul 2003 . 12924628 . 10.1078/0171-9335-00325 .
• Book: Muntau AC, Roscher AA, Kunau WH, Dodt G . Interaction of PEX3 and PEX19 Visualized by Fluorescence Resonance Energy Transfer (FRET) . Advances in Experimental Medicine and Biology . 2003 . Peroxisomal Disorders and Regulation of Genes . 544 . 221–4 . 14713233 . 10.1007/978-1-4419-9072-3_27 . 978-1-4613-4782-8 .
• Fang Y, Morrell JC, Jones JM, Gould SJ . 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 . Mar 2004 . 15007061 . 2172291 . 10.1083/jcb.200311131 .
• 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 . Towards a proteome-scale map of the human protein-protein interaction network . Nature . 437 . 7062 . 1173–8 . Oct 2005 . 16189514 . 10.1038/nature04209 . 2005Natur.437.1173R . 4427026 .
• Matsuzono Y, Matsuzaki T, Fujiki Y . Functional domain mapping of peroxin Pex19p: interaction with Pex3p is essential for function and translocation . Journal of Cell Science . 119 . Pt 17 . 3539–50 . Sep 2006 . 16895967 . 10.1242/jcs.03100 . free .

External links

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

Notes and References

1. Kammerer S, Holzinger A, Welsch U, Roscher AA . Cloning and characterization of the gene encoding the human peroxisomal assembly protein Pex3p . FEBS Letters . 429 . 1 . 53–60 . Jun 1998 . 9657383 . 10.1016/S0014-5793(98)00557-2 . 33291739 .
2. Web site: 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 . Towards a proteome-scale map of the human protein-protein interaction network . Huda Zoghbi. Nature . 437 . 7062 . 1173–8 . Oct 2005 . 16189514 . 10.1038/nature04209 . 2005Natur.437.1173R . 4427026 .
4. Mayerhofer PU, Kattenfeld T, Roscher AA, Muntau AC . Two splice variants of human PEX19 exhibit distinct functions in peroxisomal assembly . Biochemical and Biophysical Research Communications . 291 . 5 . 1180–6 . Mar 2002 . 11883941 . 10.1006/bbrc.2002.6568 .
5. Sacksteder KA, Jones JM, South ST, Li X, Liu Y, Gould SJ . 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 . Mar 2000 . 10704444 . 2174547 . 10.1083/jcb.148.5.931 .
6. Fransen M, Wylin T, Brees C, Mannaerts GP, Van Veldhoven PP . Human pex19p binds peroxisomal integral membrane proteins at regions distinct from their sorting sequences . Molecular and Cellular Biology . 21 . 13 . 4413–24 . Jul 2001 . 11390669 . 87101 . 10.1128/MCB.21.13.4413-4424.2001 .