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Gene Review

PEX14  -  peroxisomal biogenesis factor 14

Homo sapiens

Synonyms: NAPP2, PBD13A, PTS1 receptor-docking protein, Peroxin-14, Peroxisomal membrane anchor protein PEX14, ...
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High impact information on PEX14

  • We have used double-stranded RNA interference to target the PEX14 transcript for degradation [1].
  • Here we demonstrate that Pex13 directly binds Pex14 not only via its SH3 domain but also via a novel intraperoxisomal site [2].
  • The core protein of this complex, Pex14, is thought to represent the initial docking site for the import receptors Pex5 and Pex7 [2].
  • Peroxisome function was affected only mildly by mutations within the novel Pex14 interaction site of Pex13 or by the non-Pex13-interacting mutant Pex5(W204A) [2].
  • Based on two-hybrid analyses in mammalian cells and complementary in vitro binding assays, we demonstrate that the evolutionarily conserved pentapeptide repeat motifs, WX(E/D/Q/A/S)(E/D/Q)(F/Y), in PEX5 bind to PEX14 with high affinity [3].

Biological context of PEX14


Anatomical context of PEX14

  • Furthermore, we showed that the patient's fibroblasts lacked PEX14 as determined by immunocytochemical analysis [5].
  • Although PEX14 mutants have been identified in yeasts and CHO-cells, human PEX14 deficiency has apparently not been documented [5].
  • The low sequence identities of PEX14 and PEX5 between parasite and its human host, and the vital importance of proper glycosome biogenesis to the parasite, render these peroxins highly promising drug targets [8].

Associations of PEX14 with chemical compounds


Other interactions of PEX14


Analytical, diagnostic and therapeutic context of PEX14


  1. Glucose is toxic to glycosome-deficient trypanosomes. Furuya, T., Kessler, P., Jardim, A., Schnaufer, A., Crudder, C., Parsons, M. Proc. Natl. Acad. Sci. U.S.A. (2002) [Pubmed]
  2. Identification of a novel, intraperoxisomal pex14-binding site in pex13: association of pex13 with the docking complex is essential for peroxisomal matrix protein import. Schell-Steven, A., Stein, K., Amoros, M., Landgraf, C., Volkmer-Engert, R., Rottensteiner, H., Erdmann, R. Mol. Cell. Biol. (2005) [Pubmed]
  3. The di-aromatic pentapeptide repeats of the human peroxisome import receptor PEX5 are separate high affinity binding sites for the peroxisomal membrane protein PEX14. Saidowsky, J., Dodt, G., Kirchberg, K., Wegner, A., Nastainczyk, W., Kunau, W.H., Schliebs, W. J. Biol. Chem. (2001) [Pubmed]
  4. Recombinant human peroxisomal targeting signal receptor PEX5. Structural basis for interaction of PEX5 with PEX14. Schliebs, W., Saidowsky, J., Agianian, B., Dodt, G., Herberg, F.W., Kunau, W.H. J. Biol. Chem. (1999) [Pubmed]
  5. Identification of a new complementation group of the peroxisome biogenesis disorders and PEX14 as the mutated gene. Shimozawa, N., Tsukamoto, T., Nagase, T., Takemoto, Y., Koyama, N., Suzuki, Y., Komori, M., Osumi, T., Jeannette, G., Wanders, R.J., Kondo, N. Hum. Mutat. (2004) [Pubmed]
  6. Method for efficient transfection of in vitro-transcribed mRNA into SK-N-AS and HEK293 cells: difference in the toxicity of nuclear EGFP compared to cytoplasmic EGFP. Ejeskär, K., Fransson, S., Zaibak, F., Ioannou, P.A. Int. J. Mol. Med. (2006) [Pubmed]
  7. NAPP2, a peroxisomal membrane protein, is also a transcriptional corepressor. Gavva, N.R., Wen, S.C., Daftari, P., Moniwa, M., Yang, W.M., Yang-Feng, L.P., Seto, E., Davie, J.R., Shen, C.K. Genomics (2002) [Pubmed]
  8. Analysis of the sequence motifs responsible for the interactions of peroxins 14 and 5, which are involved in glycosome biogenesis in Trypanosoma brucei. Choe, J., Moyersoen, J., Roach, C., Carter, T.L., Fan, E., Michels, P.A., Hol, W.G. Biochemistry (2003) [Pubmed]
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