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PEX26 belongs to the peroxin-26 gene family. Additionally we are shipping PEX26 Antibodies (26) and many more products for this protein.
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In yeast, PEX26 follows the pathway that also ensures correct targeting of Pex15: PEX26 enters the endoplasmic reticulum (ER) in a GET-dependent and Pex19 (show PEX19 Proteins)-independent manner.
results suggested that peroxisome biogenesis requires Pex1p- and Pex6p-regulated dissociation of Pex14p (show PEX14 Proteins) from Pex26p
PEX19 (show PEX19 Proteins) formed a complex with the peroxisomal tail anchored protein PEX26 in the cytosol and translocated it directly to peroxisomes by a TRC40 (show ASNA1 Proteins)-independent class I pathway.
degree of temperature sensitivity in pex26 cell lines is predictive of the clinical phenotype in patients with PEX26 deficiency
PEX26 deficiency impairs peroxisomal import of both PTS1- and PTS2-targeted matrix proteins. It undergoes alternative splicing to produce several splice forms, including PEX26-delta, which rescues peroxisome biogenesis in PEX26-deficient cells.
Pex26p functions in recruiting to peroxisomes the complexes of the AAA ATPase peroxins.
We analyzed targeting of human PEX26. Its C-terminal-targeting signal contains two binding sites for PEX19 (show PEX19 Proteins) and we conclude C-terminal PEX19 (show PEX19 Proteins)-binding sites mark tail-anchored proteins for delivery to peroxisomes.
the relative fraction of disease-causing alleles that occur in the coding and splice junction sequences of PEX26 gene.
This gene belongs to the peroxin-26 gene family. It is probably required for protein import into peroxisomes. It anchors PEX1 and PEX6 to peroxisome membranes, possibly to form heteromeric AAA ATPase complexes required for the import of proteins into peroxisomes. Defects in this gene are the cause of peroxisome biogenesis disorder complementation group 8 (PBD-CG8). PBD refers to a group of peroxisomal disorders arising from a failure of protein import into the peroxisomal membrane or matrix. The PBD group is comprised of four disorders: Zellweger syndrome (ZWS), neonatal adrenoleukodystrophy (NALD), infantile Refsum disease (IRD), and classical rhizomelic chondrodysplasia punctata (RCDP). Alternatively spliced transcript variants have been identified for this gene.
peroxisome assembly protein 26
, peroxisome biogenesis factor 26
, peroxisome biogenesis disorder, complementation group 8
, peroxisome biogenesis disorder, complementation group A