Conjugation: HRP: Horseradish Peroxidase
Description of Target: PEX5 binds to the C-terminal PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import. Peroxins (PEXs) are proteins that are essential for the assembly of functional peroxisomes. The peroxisome biogenesis disorders (PBDs) are a group of genetically heterogeneous autosomal recessive, lethal diseases characterized by multiple defects in peroxisome function. The peroxisomal biogenesis disorders are a heterogeneous group with at least 14 complementation groups and with more than 1 phenotype being observed in cases falling into particular complementation groups. Although the clinical features of PBD patients vary, cells from all PBD patients exhibit a defect in the import of one or more classes of peroxisomal matrix proteins into the organelle. Defects in this gene are a cause of neonatal adrenoleukodystrophy (NALD), a cause of Zellweger syndrome (ZWS) as well as may be a cause of infantile Refsum disease (IRD).
Immunogen: The immunogen is a synthetic peptide directed towards the N terminal region of human PEX5
Key Reference: Maynard,E.L. (2007) J. Mol. Biol. 368 (5), 1259-1266
Molecular Weight: 70kDa
Peptide Sequence: Synthetic peptide located within the following region:
TATDRWYDEYHPEEDLQHTASDFVAKVDDPKLANSEFLKFVRQIGEGQVSProduct Format: Liquid. Purified antibody is supplied in high phosphate PBS, 100 mm phosphate, 150 mM NaCl, pH 7.6.
Protein Name: Peroxisomal targeting signal 1 receptor
Protein Size: 631
Purification: Affinity Purified
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