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Data suggest that soluble/cytosolic PEX5 (show PEX5 Proteins) interacts with PEX14 (show PEX14 Proteins)/PEX13 complex, a model for the docking/translocation module (DTM) of the peroxisomal matrix protein translocon; PEX14 (show PEX14 Proteins)/PEX13 complex appears to function in peroxisomal membrane as large cavity into which cytosolic PEX5 (show PEX5 Proteins) can enter to release its cargo. (PEX (show PHEX Proteins) = peroxisomal biogenesis factor)
our results demonstrate that PEX13 is required for selective autophagy, and suggest that dysregulation of PEX13-mediated mitophagy may contribute to ZSS pathogenesis.
Results show PEX13 interacts with itself in peroxisomes in living cells and the import of PTS1 (peroxisomal targeting signal 1) proteins is specifically disrupted when homooligomerization of PEX13 is interrupted.
Pex13p has a role in determining the peroxisomal localization of Pex14p (show PEX14 Proteins)
analysis of PEX13 substitution of Ile326 by threonine in a patient with peroxisomal biogenesis disorder [case report]
Zellweger syndrome (show PEX1 Proteins) caused by PEX13 deficiency: report of two novel mutations.
PEX13 deficiency leads to mitochondria-mediated oxidative stress, neuronal cell death and impairment of cerebellar development.
pex13 has a role in peroxisome biogenesis and its disruption in mice causes a Zellweger syndrome (show PEX1 Proteins) phenotype
This gene encodes a peroxisomal membrane protein that binds the type 1 peroxisomal targeting signal receptor via a SH3 domain located in the cytoplasm. Mutations and deficiencies in peroxisomal protein importing and peroxisome assembly lead to peroxisomal biogenesis disorders, an example of which is Zellweger syndrome.
, peroxin 13
, peroxisomal membrane protein PEX13
, peroxisome biogenesis factor 13
, potential peroxisomal membrane protein Pex13
, peroxisomal biogenesis factor 13
, transmembrane protein
, putative peroxin 13
, Peroxisomal membrane protein PEX13
, peroxisomal membrane protein PEX13-like