PEX19 explained

Peroxisomal biogenesis factor 19 is a protein that in humans is encoded by the PEX19 gene.^{[1] [2] [3]}

Interactions

PEX19 has been shown to interact with:

• ABCD1,
• ABCD2,
• ABCD3,^{[4] [5] [6]}
• PEX10,^{[7] [8]}
• PEX11B,^{[7] [8]}
• PEX12,^{[7] [8]}
• PEX13,^{[7] [8]}
• PEX16,^[8] and
• PEX3.^{[4] [7] [8] [9]}

Further reading

• Braun A, Kammerer S, Weissenhorn W, Weiss EH, Cleve H . Sequence of a putative human housekeeping gene (HK33) localized on chromosome 1 . Gene . 146 . 2 . 291–5 . 1994 . 8076834 . 10.1016/0378-1119(94)90308-5 .
• Götte K, Girzalsky W, Linkert M, Baumgart E, Kammerer S, Kunau WH, Erdmann R . Pex19p, a Farnesylated Protein Essential for Peroxisome Biogenesis . Mol. Cell. Biol. . 18 . 1 . 616–28 . 1998 . 9418908 . 121529 . 10.1128/MCB.18.1.616.
• 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 . J. Cell Biol. . 148 . 5 . 931–44 . 2000 . 10704444 . 2174547 . 10.1083/jcb.148.5.931 .
• Gloeckner CJ, Mayerhofer PU, Landgraf P, Muntau AC, Holzinger A, Gerber JK, Kammerer S, Adamski J, Roscher AA . Human adrenoleukodystrophy protein and related peroxisomal ABC transporters interact with the peroxisomal assembly protein PEX19p . Biochem. Biophys. Res. Commun. . 271 . 1 . 144–50 . 2000 . 10777694 . 10.1006/bbrc.2000.2572 .
• Sugihara T, Kaul SC, Kato J, Reddel RR, Nomura H, Wadhwa R . Pex19p dampens the p19ARF-p53-p21WAF1 tumor suppressor pathway . J. Biol. Chem. . 276 . 22 . 18649–52 . 2001 . 11259404 . 10.1074/jbc.C100011200 . free .
• 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 . Mol. Cell. Biol. . 21 . 13 . 4413–24 . 2001 . 11390669 . 87101 . 10.1128/MCB.21.13.4413-4424.2001 .
• Biermanns M, Gärtner J . Targeting elements in the amino-terminal part direct the human 70-kDa peroxisomal integral membrane protein (PMP70) to peroxisomes . Biochem. Biophys. Res. Commun. . 285 . 3 . 649–55 . 2001 . 11453642 . 10.1006/bbrc.2001.5220 .
• Mayerhofer PU, Kattenfeld T, Roscher AA, Muntau AC . Two splice variants of human PEX19 exhibit distinct functions in peroxisomal assembly . Biochem. Biophys. Res. Commun. . 291 . 5 . 1180–6 . 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 . Mol. Cell. Proteomics . 1 . 3 . 243–52 . 2002 . 12096124 . 10.1074/mcp.M100025-MCP200 . free .
• Ito M, Iidawa S, Izuka M, Haito S, Segawa H, Kuwahata M, Ohkido I, Ohno H, Miyamoto K . Interaction of a farnesylated protein with renal type IIa Na/Pi co-transporter in response to parathyroid hormone and dietary phosphate . Biochem. J. . 377 . Pt 3 . 607–16 . 2004 . 14558883 . 1223893 . 10.1042/BJ20031223 .
• Jones JM, Morrell JC, Gould SJ . PEX19 is a predominantly cytosolic chaperone and import receptor for class 1 peroxisomal membrane proteins . J. Cell Biol. . 164 . 1 . 57–67 . 2004 . 14709540 . 2171958 . 10.1083/jcb.200304111 .
• Book: Muntau AC, Roscher AA, Kunau WH, Dodt G . Interaction of PEX3 and PEX19 Visualized by Fluorescence Resonance Energy Transfer (FRET) . Peroxisomal Disorders and Regulation of Genes . 544 . 221–4 . 14713233 . 10.1007/978-1-4419-9072-3_27 . Advances in Experimental Medicine and Biology . 2003 . 978-1-4613-4782-8 .
• Fransen M, Vastiau I, Brees C, Brys V, Mannaerts GP, Van Veldhoven PP . Potential role for Pex19p in assembly of PTS-receptor docking complexes . J. Biol. Chem. . 279 . 13 . 12615–24 . 2004 . 14715663 . 10.1074/jbc.M304941200 . free .
• Fang Y, Morrell JC, Jones JM, Gould SJ . PEX3 functions as a PEX19 docking factor in the import of class I peroxisomal membrane proteins . J. Cell Biol. . 164 . 6 . 863–75 . 2004 . 15007061 . 2172291 . 10.1083/jcb.200311131 .
• Colland F, Jacq X, Trouplin V, Mougin C, Groizeleau C, Hamburger A, Meil A, Wojcik J, Legrain P, Gauthier JM . Functional Proteomics Mapping of a Human Signaling Pathway . Genome Res. . 14 . 7 . 1324–32 . 2004 . 15231748 . 442148 . 10.1101/gr.2334104 .
• Shibata H, Kashiwayama Y, Imanaka T, Kato H . Domain architecture and activity of human Pex19p, a chaperone-like protein for intracellular trafficking of peroxisomal membrane proteins . J. Biol. Chem. . 279 . 37 . 38486–94 . 2004 . 15252024 . 10.1074/jbc.M402204200 . 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, Arnold N, Gutensohn W, Mewes HW, Kunau WH, Höfler G, Roscher AA, Braun A . Genomic organization and molecular characterization of a gene encoding HsPXF, a human peroxisomal farnesylated protein . Genomics . 45 . 1 . 200–10 . November 1997 . 9339377 . 10.1006/geno.1997.4914 .
2. Matsuzono Y, Kinoshita N, Tamura S, Shimozawa N, Hamasaki M, Ghaedi K, Wanders RJ, Suzuki Y, Kondo N, Fujiki Y . Human PEX19: cDNA cloning by functional complementation, mutation analysis in a patient with Zellweger syndrome, and potential role in peroxisomal membrane assembly . Proc Natl Acad Sci U S A . 96 . 5 . 2116–21 . April 1999 . 10051604 . 26746 . 10.1073/pnas.96.5.2116 . 1999PNAS...96.2116M . free .
3. Web site: Entrez Gene: PEX19 peroxisomal biogenesis factor 19.
4. Mayerhofer PU, Kattenfeld T, Roscher AA, Muntau AC . Two splice variants of human PEX19 exhibit distinct functions in peroxisomal assembly . Biochem. Biophys. Res. Commun. . 291 . 5 . 1180–6 . March 2002 . 11883941 . 10.1006/bbrc.2002.6568 .
5. Gloeckner CJ, Mayerhofer PU, Landgraf P, Muntau AC, Holzinger A, Gerber JK, Kammerer S, Adamski J, Roscher AA . Human adrenoleukodystrophy protein and related peroxisomal ABC transporters interact with the peroxisomal assembly protein PEX19p . Biochem. Biophys. Res. Commun. . 271 . 1 . 144–50 . April 2000 . 10777694 . 10.1006/bbrc.2000.2572 .
6. Biermanns M, Gärtner J . Targeting elements in the amino-terminal part direct the human 70-kDa peroxisomal integral membrane protein (PMP70) to peroxisomes . Biochem. Biophys. Res. Commun. . 285 . 3 . 649–55 . July 2001 . 11453642 . 10.1006/bbrc.2001.5220 .
7. 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 . J. Cell Biol. . 148 . 5 . 931–44 . March 2000 . 10704444 . 2174547 . 10.1083/jcb.148.5.931.
8. 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 . Mol. Cell. Biol. . 21 . 13 . 4413–24 . July 2001 . 11390669 . 87101 . 10.1128/MCB.21.13.4413-4424.2001 .
9. 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 . October 2005 . 16189514 . 10.1038/nature04209 . 2005Natur.437.1173R . 4427026 .