PEX5 Antibody - N-terminal region : Biotin (ARP56101_P050-Biotin)

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) .

CAPRIN2; CCDC14; TOMM7; PRR13; GDPD5; ZNF772; EP400NL; TM6SF1; ACOT8; RPL14; MKRN3; S100A6; LDHB; RANBP6; GSTK1; ZADH2; TYSND1; HSDL2; ACAD11; LONP2; PECR; HSD17B4; HOXA7; EHHADH; ECH1; CRAT; CAT; BRCA1; ACOX1; DECR2; HACL1; DHRS4; ECI2; AGPS; GNPAT; UBC

All conjugated antibodies should be stored in light-protected vials or covered with a light protecting material (i.e. aluminum foil) . Conjugated antibodies are stable for at least 12 months at 4C. If longer storage is desired (24 months), conjugates may be diluted with up to 50% glycerol and stored at -20C to -80C. Freezing and thawing conjugated antibodies will compromise enzyme activity as well as antibody binding.