PEX14 explained

Peroxisomal membrane protein PEX14 is a protein that in humans is encoded by the PEX14 gene.^{[1] [2]}

Function

This gene encodes an essential component of the peroxisomal import machinery. The protein is integrated into peroxisome membranes with its C-terminus exposed to the cytosol, and interacts with the cytosolic receptor for proteins containing a PTS1 peroxisomal targeting signal. The protein also functions as a transcriptional corepressor and interacts with a histone deacetylase. A mutation in this gene results in one form of Zellweger syndrome.

Interactions

PEX14 has been shown to interact with

• PEX5,^{[3] [4] [5] [6]}
• PEX7,^[6] and
• PEX13.^[3]

Further reading

• Albertini M, Rehling P, Erdmann R, Girzalsky W, Kiel JA, Veenhuis M, Kunau WH . Pex14p, a peroxisomal membrane protein binding both receptors of the two PTS-dependent import pathways . Cell . 89 . 1 . 83–92 . Apr 1997 . 9094717 . 10.1016/S0092-8674(00)80185-3 . 18878596 .
• Huhse B, Rehling P, Albertini M, Blank L, Meller K, Kunau WH . Pex17p of Saccharomyces cerevisiae is a novel peroxin and component of the peroxisomal protein translocation machinery . The Journal of Cell Biology . 140 . 1 . 49–60 . Jan 1998 . 9425153 . 2132588 . 10.1083/jcb.140.1.49 .
• Will GK, Soukupova M, Hong X, Erdmann KS, Kiel JA, Dodt G, Kunau WH, Erdmann R . Identification and characterization of the human orthologue of yeast Pex14p . Molecular and Cellular Biology . 19 . 3 . 2265–77 . Mar 1999 . 10022913 . 84019 . 10.1128/mcb.19.3.2265.
• Girzalsky W, Rehling P, Stein K, Kipper J, Blank L, Kunau WH, Erdmann R . Involvement of Pex13p in Pex14p localization and peroxisomal targeting signal 2-dependent protein import into peroxisomes . The Journal of Cell Biology . 144 . 6 . 1151–62 . Mar 1999 . 10087260 . 2150583 . 10.1083/jcb.144.6.1151 .
• Shimizu N, Itoh R, Hirono Y, Otera H, Ghaedi K, Tateishi K, Tamura S, Okumoto K, Harano T, Mukai S, Fujiki Y . 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 . Apr 1999 . 10212238 . 10.1074/jbc.274.18.12593 . free .
• 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 .
• Gavva NR, Wen SC, Daftari P, Moniwa M, Yang WM, Yang-Feng LP, Seto E, Davie JR, Shen CK . NAPP2, a peroxisomal membrane protein, is also a transcriptional corepressor . Genomics . 79 . 3 . 423–31 . Mar 2002 . 11863372 . 10.1006/geno.2002.6714 .
• Otera H, Setoguchi K, Hamasaki M, Kumashiro T, Shimizu N, Fujiki Y . 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 . Mar 2002 . 11865044 . 135613 . 10.1128/MCB.22.6.1639-1655.2002 .
• Alland L, David G, Shen-Li H, Potes J, Muhle R, Lee HC, Hou H, Chen K, DePinho RA . Identification of mammalian Sds3 as an integral component of the Sin3/histone deacetylase corepressor complex . Molecular and Cellular Biology . 22 . 8 . 2743–50 . Apr 2002 . 11909966 . 133736 . 10.1128/MCB.22.8.2743-2750.2002 .
• 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 .
• Oliveira ME, Reguenga C, Gouveia AM, Guimarães CP, Schliebs W, Kunau WH, Silva MT, Sá-Miranda C, Azevedo JE . Mammalian Pex14p: membrane topology and characterisation of the Pex14p-Pex14p interaction . Biochimica et Biophysica Acta (BBA) - Biomembranes . 1567 . 1–2 . 13–22 . Dec 2002 . 12488033 . 10.1016/s0005-2736(02)00635-1 . free .
• Fransen M, Vastiau I, Brees C, Brys V, Mannaerts GP, Van Veldhoven PP . Potential role for Pex19p in assembly of PTS-receptor docking complexes . The Journal of Biological Chemistry . 279 . 13 . 12615–24 . Mar 2004 . 14715663 . 10.1074/jbc.M304941200 . free .
• Shimozawa N, Tsukamoto T, Nagase T, Takemoto Y, Koyama N, Suzuki Y, Komori M, Osumi T, Jeannette G, Wanders RJ, Kondo N . Identification of a new complementation group of the peroxisome biogenesis disorders and PEX14 as the mutated gene . Human Mutation . 23 . 6 . 552–8 . Jun 2004 . 15146459 . 10.1002/humu.20032 . 6324241 . free .
• 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 .
• Kimura K, Wakamatsu A, Suzuki Y, Ota T, Nishikawa T, Yamashita R, Yamamoto J, Sekine M, Tsuritani K, Wakaguri H, Ishii S, Sugiyama T, Saito K, Isono Y, Irie R, Kushida N, Yoneyama T, Otsuka R, Kanda K, Yokoi T, Kondo H, Wagatsuma M, Murakawa K, Ishida S, Ishibashi T, Takahashi-Fujii A, Tanase T, Nagai K, Kikuchi H, Nakai K, Isogai T, Sugano S . Diversification of transcriptional modulation: large-scale identification and characterization of putative alternative promoters of human genes . Genome Research . 16 . 1 . 55–65 . Jan 2006 . 16344560 . 1356129 . 10.1101/gr.4039406 .
• Nguyen T, Bjorkman J, Paton BC, Crane DI . Failure of microtubule-mediated peroxisome division and trafficking in disorders with reduced peroxisome abundance . Journal of Cell Science . 119 . Pt 4 . 636–45 . Feb 2006 . 16449325 . 10.1242/jcs.02776 . free . 2440/23242 . 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. Fransen M, Terlecky SR, Subramani S . 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 . Jul 1998 . 9653144 . 20933 . 10.1073/pnas.95.14.8087 . 1998PNAS...95.8087F . free .
2. Web site: Entrez Gene: PEX14 peroxisomal biogenesis factor 14.
3. Otera H, Setoguchi K, Hamasaki M, Kumashiro T, Shimizu N, Fujiki Y . 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 . Mar 2002 . 11865044 . 135613 . 10.1128/MCB.22.6.1639-1655.2002 .
4. Okumoto K, Abe I, Fujiki Y . 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 . Aug 2000 . 10837480 . 10.1074/jbc.M003303200 . free .
5. Shimizu N, Itoh R, Hirono Y, Otera H, Ghaedi K, Tateishi K, Tamura S, Okumoto K, Harano T, Mukai S, Fujiki Y . 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 . Apr 1999 . 10212238 . 10.1074/jbc.274.18.12593 . free .
6. Managadze D, Würtz C, Sichting M, Niehaus G, Veenhuis M, Rottensteiner H . The peroxin PEX14 of Neurospora crassa is essential for the biogenesis of both glyoxysomes and Woronin bodies . Traffic . 8 . 6 . 687–701 . Jun 2007 . 17461798 . 10.1111/j.1600-0854.2007.00560.x . 221250 . free .