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Gene Review

PEX10  -  peroxisomal biogenesis factor 10

Homo sapiens

Synonyms: NALD, PBD6A, PBD6B, Peroxin-10, Peroxisomal biogenesis factor 10, ...
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Disease relevance of PEX10

  • Before OLT, median CDT values were determined to be elevated among patients with alcoholic as well as nonalcoholic end-stage liver diseases (NALD) [1].
  • The patients were divided into 4 etiological groups: alcoholic cirrhosis (ALD), (38) non-alcoholic cirrhosis (NALD) (35) non-cirrhotic portal fibrosis (NCPF) (14) and extrahepatic portal vein obstruction (EHPVO) (14) [2].

High impact information on PEX10

  • Overexpression of either PEX5 or PEX10 can suppress this PEX12 mutation, providing genetic evidence that these interactions are biologically relevant [3].
  • Furthermore, we identified a patient with a missense mutation in the PEX12 zinc-binding domain, S320F, and observed that this mutation reduces the binding of PEX12 to PEX5 and PEX10 [3].
  • PEX10-deficient PBD100 cells contain many peroxisomes and import peroxisomal membrane proteins but do not import peroxisomal matrix proteins, indicating that loss of PEX10 has its most pronounced effect on peroxisomal matrix-protein import [4].
  • We identified the human orthologue of yeast PEX10 and observed that its expression rescues peroxisomal matrix-protein import in PBD patients' fibroblasts from complementation group 7 (CG7) [4].
  • A Zellweger syndrome patient, PBD100, was homozygous for a splice donor-site mutation that results in exon skipping and loss of 407 bp from the PEX10 open reading frame [4].

Biological context of PEX10


Anatomical context of PEX10

  • However, we find that loss of PEX12 or PEX10 does not reduce the association of PEX5 with peroxisomes, demonstrating that these peroxins are not required for receptor docking [3].
  • GFP fusions of T. cruzi PEX10 and L. major PEX12 also localized to glycosomes in T. brucei indicating that glycosomal membrane protein targeting is conserved across trypanosomatids [7].
  • MDA-HSA induced PBMC T-cell proliferation was significantly higher in ALD than in NALD or control patients [8].
  • MATERIALS AND METHODS: We used immunologic phenotyping, and isolation methods to fractionate subpopulations of nonadherent, low-density (NALD) human bone marrow cells [9].

Associations of PEX10 with chemical compounds

  • Results from more recent studies of various plant PMPs including ascorbate peroxidase, PEX10 and PEX16, as well as a viral replication protein, have since led to the formulation of a more elaborate "ER semi-autonomous peroxisome maturation and replication" model [10].

Physical interactions of PEX10

  • PEX12 interacts with PEX5 and PEX10 and acts downstream of receptor docking in peroxisomal matrix protein import [3].

Other interactions of PEX10

  • These and other results lead us to propose that PEX12 and PEX10 play direct roles in peroxisomal matrix protein import downstream of the receptor docking event [3].
  • All the 11 ZS patients with group-B PBD had a common mutation, i.e., a homozygous 2-base-pair deletion in PEX10 [5].
  • A more mildly affected neonatal adrenoleukodystrophy patient was a compound heterozygote for a missense mutation in the PEX10 zinc-binding domain, H290Q, and for a nonsense mutation, R125ter [4].

Analytical, diagnostic and therapeutic context of PEX10


  1. Carbohydrate-deficient transferrin: diagnostic efficiency among patients with end-stage liver disease before and after liver transplantation. Heinemann, A., Sterneck, M., Kuhlencordt, R., Rogiers, X., Schulz, K.H., Queen, B., Wischhusen, F., Püschel, K. Alcohol. Clin. Exp. Res. (1998) [Pubmed]
  2. Significance of radioisotope bone marrow uptake on 99m technetium sulphocolloid scan in portal hypertension. Sheth, S.G., Amarapurkar, D.N., Chopra, K.B., Mehta, P.J. The Journal of the Association of Physicians of India. (1996) [Pubmed]
  3. PEX12 interacts with PEX5 and PEX10 and acts downstream of receptor docking in peroxisomal matrix protein import. Chang, C.C., Warren, D.S., Sacksteder, K.A., Gould, S.J. J. Cell Biol. (1999) [Pubmed]
  4. Identification of PEX10, the gene defective in complementation group 7 of the peroxisome-biogenesis disorders. Warren, D.S., Morrell, J.C., Moser, H.W., Valle, D., Gould, S.J. Am. J. Hum. Genet. (1998) [Pubmed]
  5. Genetic heterogeneity of peroxisome biogenesis disorders among Japanese patients: evidence for a founder haplotype for the most common PEX10 gene mutation. Shimozawa, N., Nagase, T., Takemoto, Y., Ohura, T., Suzuki, Y., Kondo, N. Am. J. Med. Genet. A (2003) [Pubmed]
  6. Mutations in PEX10 is the cause of Zellweger peroxisome deficiency syndrome of complementation group B. Okumoto, K., Itoh, R., Shimozawa, N., Suzuki, Y., Tamura, S., Kondo, N., Fujiki, Y. Hum. Mol. Genet. (1998) [Pubmed]
  7. Characterization of glycosomal RING finger proteins of trypanosomatids. Saveria, T., Kessler, P., Jensen, B.C., Parsons, M. Exp. Parasitol. (2007) [Pubmed]
  8. Oxidative stress as a trigger for cellular immune responses in patients with alcoholic liver disease. Stewart, S.F., Vidali, M., Day, C.P., Albano, E., Jones, D.E. Hepatology (2004) [Pubmed]
  9. Bone marrow accessory cells regulate human bone precursor cell development. Eipers, P.G., Kale, S., Taichman, R.S., Pipia, G.G., Swords, N.A., Mann, K.G., Long, M.W. Exp. Hematol. (2000) [Pubmed]
  10. The ER-peroxisome connection in plants: Development of the "ER semi-autonomous peroxisome maturation and replication" model for plant peroxisome biogenesis. Mullen, R.T., Trelease, R.N. Biochim. Biophys. Acta (2006) [Pubmed]
  11. Liver disease-related admissions in Portugal: clinical and demographic pattern. Cortez-Pinto, H., Marques-Vidal, P., Monteiro, E. European journal of gastroenterology & hepatology. (2004) [Pubmed]
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