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Gene Review

PEX19  -  peroxisomal biogenesis factor 19

Homo sapiens

Synonyms: 33 kDa housekeeping protein, D1S2223E, HK33, OK/SW-cl.22, PBD12A, ...
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Disease relevance of PEX19


High impact information on PEX19

  • Both human and yeast cells lacking peroxisomes due to mutations in PEX3 or PEX19 genes regenerate the organelles upon reintroduction of the corresponding wild-type version [6].
  • PEX3 functions as a PEX19 docking factor in the import of class I peroxisomal membrane proteins [7].
  • PEX19 binds these PMPs in the cytoplasm and delivers them to the peroxisome for subsequent insertion into the peroxisome membrane, indicating that there may be a PEX19 docking factor in the peroxisome membrane [7].
  • Here, we demonstrate that PEX19 binds and stabilizes newly synthesized PMPs in the cytosol, binds to multiple PMP targeting signals (mPTSs), interacts with the hydrophobic domains of PMP targeting signals, and is essential for PMP targeting and import [8].
  • In addition, we demonstrate that PEX19, a factor required for peroxisomal membrane biogenesis, interacts with the two minimal targeting regions of PMP34 [9].

Biological context of PEX19


Anatomical context of PEX19

  • We corroborated the requirement of the ALDP PEX19-binding site for peroxisomal targeting in human fibroblasts and showed that the minimal ALDP fragment targets correctly also in yeast, again in a PEX19-binding site-dependent manner [10].
  • Pex19p was partly localized to peroxisomes but mostly localized in the cytosol [13].
  • This hypothesis is supported by the observation that the loss of PEX19 results in degradation of PMPs and/or mislocalization of PMPs to the mitochondrion [14].
  • Identification of trypanosomatid PEX19: functional characterization reveals impact on cell growth and glycosome size and number [15].
  • Western blot analysis indicates that PEX19 is located in the cytosolic and brush-border membrane fractions (microvilli and the subapical component) [16].

Associations of PEX19 with chemical compounds


Physical interactions of PEX19


Regulatory relationships of PEX19

  • To address this question by fluorescence resonance energy transfer (FRET) analysis, we engineered the enhanced yellow fluorescent protein (EYFP) to the C-terminus of PEX3 and the enhanced cyan fluorescent protein (ECFP) to the N-terminus of PEX19 [23].

Other interactions of PEX19

  • PEX3, PEX16 and PEX19 are considered to be required for the early stage of peroxisome biogenesis [24].
  • Alternatively, Pex19p might act as a multifunctional peroxin and participate in a number of these activities [20].
  • Human PEX19: cDNA cloning by functional complementation, mutation analysis in a patient with Zellweger syndrome, and potential role in peroxisomal membrane assembly [25].
  • A highly conserved N-terminal region, encompassing residues 21-70, interacts with the PTS1 receptor Pex5p, Pex13p, and Pex19p that is essential for membrane biogenesis [26].
  • To further examine the molecular mechanism underlying this competition, six evolutionarily conserved amino acids in the Pex5p/Pex13p/Pex19p binding domain of Pex14p were subjected to site-directed mutagenesis and the corresponding mutants functionally analyzed [27].

Analytical, diagnostic and therapeutic context of PEX19


  1. Domain architecture and activity of human Pex19p, a chaperone-like protein for intracellular trafficking of peroxisomal membrane proteins. Shibata, H., Kashiwayama, Y., Imanaka, T., Kato, H. J. Biol. Chem. (2004) [Pubmed]
  2. Intermittent claudication in diabetics: treatment with exercise and pentoxifylline--a 6-month, controlled, randomized trial. Belcaro, G., Nicolaides, A.N., Griffin, M., De Sanctis, M.T., Cesarone, M.R., Incandela, L., Ippolito, E., Pomante, P., Geroulakos, G., Ramaswami, G. Angiology. (2002) [Pubmed]
  3. Treatment of venous ulcers with pentoxifylline: a 6-month randomized, double-blind, placebo controlled trial. Belcaro, G., Cesarone, M.R., Nicolaides, A.N., De Sanctis, M.T., Incandela, L., Geroulakos, G. Angiology. (2002) [Pubmed]
  4. Argon versus selective laser trabeculoplasty. Van de Veire, S., Zeyen, T., Stalmans, I. Bulletin de la Société belge d'ophtalmologie. (2006) [Pubmed]
  5. Treatment of retinal vein thrombosis with pentoxifylline: a controlled, randomized trial. De Sanctis, M.T., Cesarone, M.R., Belcaro, G., Incandela, L., Steigerwalt, R., Nicolaides, A.N., Griffin, M., Geroulakos, G. Angiology. (2002) [Pubmed]
  6. Contribution of the endoplasmic reticulum to peroxisome formation. Hoepfner, D., Schildknegt, D., Braakman, I., Philippsen, P., Tabak, H.F. Cell (2005) [Pubmed]
  7. PEX3 functions as a PEX19 docking factor in the import of class I peroxisomal membrane proteins. Fang, Y., Morrell, J.C., Jones, J.M., Gould, S.J. J. Cell Biol. (2004) [Pubmed]
  8. PEX19 is a predominantly cytosolic chaperone and import receptor for class 1 peroxisomal membrane proteins. Jones, J.M., Morrell, J.C., Gould, S.J. J. Cell Biol. (2004) [Pubmed]
  9. Multiple distinct targeting signals in integral peroxisomal membrane proteins. Jones, J.M., Morrell, J.C., Gould, S.J. J. Cell Biol. (2001) [Pubmed]
  10. Function of the PEX19-binding site of human adrenoleukodystrophy protein as targeting motif in man and yeast. PMP targeting is evolutionarily conserved. Halbach, A., Lorenzen, S., Landgraf, C., Volkmer-Engert, R., Erdmann, R., Rottensteiner, H. J. Biol. Chem. (2005) [Pubmed]
  11. Two splice variants of human PEX19 exhibit distinct functions in peroxisomal assembly. Mayerhofer, P.U., Kattenfeld, T., Roscher, A.A., Muntau, A.C. Biochem. Biophys. Res. Commun. (2002) [Pubmed]
  12. Interaction of PEX3 and PEX19 visualized by fluorescence resonance energy transfer (FRET). Muntau, A.C., Roscher, A.A., Kunau, W.H., Dodt, G. Adv. Exp. Med. Biol. (2003) [Pubmed]
  13. Functional domain mapping of peroxin Pex19p: interaction with Pex3p is essential for function and translocation. Matsuzono, Y., Matsuzaki, T., Fujiki, Y. J. Cell. Sci. (2006) [Pubmed]
  14. PEX19 binds multiple peroxisomal membrane proteins, is predominantly cytoplasmic, and is required for peroxisome membrane synthesis. Sacksteder, K.A., Jones, J.M., South, S.T., Li, X., Liu, Y., Gould, S.J. J. Cell Biol. (2000) [Pubmed]
  15. Identification of trypanosomatid PEX19: functional characterization reveals impact on cell growth and glycosome size and number. Banerjee, S.K., Kessler, P.S., Saveria, T., Parsons, M. Mol. Biochem. Parasitol. (2005) [Pubmed]
  16. Interaction of a farnesylated protein with renal type IIa Na/Pi co-transporter in response to parathyroid hormone and dietary phosphate. Ito, M., Iidawa, S., Izuka, M., Haito, S., Segawa, H., Kuwahata, M., Ohkido, I., Ohno, H., Miyamoto, K. Biochem. J. (2004) [Pubmed]
  17. Targeting of the tail-anchored peroxisomal membrane proteins PEX26 and PEX15 occurs through C-terminal PEX19-binding sites. Halbach, A., Landgraf, C., Lorenzen, S., Rosenkranz, K., Volkmer-Engert, R., Erdmann, R., Rottensteiner, H. J. Cell. Sci. (2006) [Pubmed]
  18. Pex19p, a farnesylated protein essential for peroxisome biogenesis. Götte, K., Girzalsky, W., Linkert, M., Baumgart, E., Kammerer, S., Kunau, W.H., Erdmann, R. Mol. Cell. Biol. (1998) [Pubmed]
  19. Stress chaperones, mortalin, and pex19p mediate 5-aza-2' deoxycytidine-induced senescence of cancer cells by DNA methylation-independent pathway. Widodo, N., Deocaris, C.C., Kaur, K., Hasan, K., Yaguchi, T., Yamasaki, K., Sugihara, T., Ishii, T., Wadhwa, R., Kaul, S.C. J. Gerontol. A Biol. Sci. Med. Sci. (2007) [Pubmed]
  20. Analysis of human Pex19p's domain structure by pentapeptide scanning mutagenesis. Fransen, M., Vastiau, I., Brees, C., Brys, V., Mannaerts, G.P., Van Veldhoven, P.P. J. Mol. Biol. (2005) [Pubmed]
  21. Targeting elements in the amino-terminal part direct the human 70-kDa peroxisomal integral membrane protein (PMP70) to peroxisomes. Biermanns, M., Gärtner, J. Biochem. Biophys. Res. Commun. (2001) [Pubmed]
  22. Human adrenoleukodystrophy protein and related peroxisomal ABC transporters interact with the peroxisomal assembly protein PEX19p. Gloeckner, C.J., Mayerhofer, P.U., Landgraf, P., Muntau, A.C., Holzinger, A., Gerber, J.K., Kammerer, S., Adamski, J., Roscher, A.A. Biochem. Biophys. Res. Commun. (2000) [Pubmed]
  23. The interaction between human PEX3 and PEX19 characterized by fluorescence resonance energy transfer (FRET) analysis. Muntau, A.C., Roscher, A.A., Kunau, W.H., Dodt, G. Eur. J. Cell Biol. (2003) [Pubmed]
  24. Clinical, biochemical and genetic aspects and neuronal migration in peroxisome biogenesis disorders. Suzuki, Y., Shimozawa, N., Imamura, A., Fukuda, S., Zhang, Z., Orii, T., Kondo, N. J. Inherit. Metab. Dis. (2001) [Pubmed]
  25. Human PEX19: cDNA cloning by functional complementation, mutation analysis in a patient with Zellweger syndrome, and potential role in peroxisomal membrane assembly. Matsuzono, Y., Kinoshita, N., Tamura, S., Shimozawa, N., Hamasaki, M., Ghaedi, K., Wanders, R.J., Suzuki, Y., Kondo, N., Fujiki, Y. Proc. Natl. Acad. Sci. U.S.A. (1999) [Pubmed]
  26. Functional domains and dynamic assembly of the peroxin Pex14p, the entry site of matrix proteins. Itoh, R., Fujiki, Y. J. Biol. Chem. (2006) [Pubmed]
  27. Potential role for Pex19p in assembly of PTS-receptor docking complexes. Fransen, M., Vastiau, I., Brees, C., Brys, V., Mannaerts, G.P., Van Veldhoven, P.P. J. Biol. Chem. (2004) [Pubmed]
  28. Role of Pex19p in the targeting of PMP70 to peroxisome. Kashiwayama, Y., Asahina, K., Shibata, H., Morita, M., Muntau, A.C., Roscher, A.A., Wanders, R.J., Shimozawa, N., Sakaguchi, M., Kato, H., Imanaka, T. Biochim. Biophys. Acta (2005) [Pubmed]
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