Gene Symbol: Pex7
Description: peroxisomal biogenesis factor 7
Alias: MmPEX7, peroxisomal biogenesis factor 7, PTS2 receptor, peroxin-7, peroxisomal targeting signal 2 receptor, peroxisome biogenesis factor 7
Species: mouse
Products:     Pex7

Top Publications

  1. Braverman N, Steel G, Obie C, Moser A, Moser H, Gould S, et al. Human PEX7 encodes the peroxisomal PTS2 receptor and is responsible for rhizomelic chondrodysplasia punctata. Nat Genet. 1997;15:369-76 pubmed
    ..b>PEX7, a candidate gene for RCDP identified in yeast, encodes the receptor for peroxisomal matrix proteins with the type-..
  2. Brites P, Motley A, Gressens P, Mooyer P, Ploegaert I, Everts V, et al. Impaired neuronal migration and endochondral ossification in Pex7 knockout mice: a model for rhizomelic chondrodysplasia punctata. Hum Mol Genet. 2003;12:2255-67 pubmed
    ..The disorder is caused by mutations in the PEX7 gene, which encodes the receptor for a class of peroxisomal matrix enzymes...
  3. Mukai S, Ghaedi K, Fujiki Y. Intracellular localization, function, and dysfunction of the peroxisome-targeting signal type 2 receptor, Pex7p, in mammalian cells. J Biol Chem. 2002;277:9548-61 pubmed publisher
    ..Herein we have cloned Chinese hamster (Cl) PEX7 encoding the PTS2 receptor. ClPex7p consists of 318 amino acids, shorter than human Pex7p by 5 residues, showing 91 and 30% identity with ..
  4. Braverman N, Zhang R, Chen L, Nimmo G, Scheper S, Tran T, et al. A Pex7 hypomorphic mouse model for plasmalogen deficiency affecting the lens and skeleton. Mol Genet Metab. 2010;99:408-16 pubmed publisher
    ..It is a rare autosomal recessive disorder, caused by defects in the peroxisome receptor, PEX7. The pathology results from a deficiency of plasmalogens, a critical class of ether phospholipids whose functions ..
  5. Mizuno Y, Ninomiya Y, Nakachi Y, Iseki M, Iwasa H, Akita M, et al. Tysnd1 deficiency in mice interferes with the peroxisomal localization of PTS2 enzymes, causing lipid metabolic abnormalities and male infertility. PLoS Genet. 2013;9:e1003286 pubmed publisher
    ..Our data established that peroxisomal processing protease Tysnd1 is necessary to mediate the physiological functions of PTS2-containing substrates. ..
  6. Loughran P, Stolz D, Barrick S, Wheeler D, Friedman P, Rachubinski R, et al. PEX7 and EBP50 target iNOS to the peroxisome in hepatocytes. Nitric Oxide. 2013;31:9-19 pubmed publisher
    ..for iNOS localization to the peroxisome is through the interaction with peroxisomal import proteins PEX5 or PEX7. siRNA knockdown of PEX7 reduced iNOS colocalization with the peroxisomal protein PMP70...
  7. Lodhi I, Semenkovich C. Peroxisomes: a nexus for lipid metabolism and cellular signaling. Cell Metab. 2014;19:380-92 pubmed publisher
    ..Here, we review the biology of peroxisomes and their potential relevance to human disorders including cancer, obesity-related diabetes, and degenerative neurologic disease. ..
  8. da Silva T, Eira J, Lopes A, Malheiro A, Sousa V, Luoma A, et al. Peripheral nervous system plasmalogens regulate Schwann cell differentiation and myelination. J Clin Invest. 2014;124:2560-70 pubmed publisher
    ..In addition, these studies identify a mechanism by which the lack of a membrane phospholipid causes neuropathology, implicating plasmalogens as regulators of membrane and cell signaling. ..
  9. Rodrigues T, Alencastre I, Francisco T, Brites P, Fransen M, Grou C, et al. A PEX7-centered perspective on the peroxisomal targeting signal type 2-mediated protein import pathway. Mol Cell Biol. 2014;34:2917-28 pubmed publisher
    ..1 signal are carried to the peroxisome by PEX5, whereas those harboring a type 2 signal are transported by a PEX5-PEX7 complex. The pathway followed by PEX5 during the protein transport cycle has been characterized in detail...