The product of PEX3 is involved in peroxisome biosynthesis and integrity. It assembles membrane vesicles before the matrix proteins are translocated. 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 PEX3 are a cause Zellweger syndrome (ZWS).蛋白别名为:PEX3; Peroxisomal biogenesis factor 3; Peroxin-3; Peroxisomal assembly protein PEX3;基因ID为:8504;蛋白质ID:P56589
应用类型
WB,ELISA补充:最优的抗体稀释比例需要基于客户实验进行优化.建议的起始稀释比例如下: WB: 1:500-1:2000, ELISA: 1:10000. Not yet tested in other applications.
免疫原
合成多肽:the N-terminal region of human Peroxin 3. at AA rangle: 30-110