Anti-PEX5 Polyclonal Antibody-一抗-抗体-蛋白与免疫

Anti-PEX5 Polyclonal Antibody

品牌:solarbio | 货号:K004208P

英文名称 Anti-PEX5 Polyclonal Antibody
宿主 Rabbit
别名 PBD2A;PBD2B;PTS1-BP;PTS1R;PXR1;RCDP5
应用 WB IHC IF
稀释比例 WB 1:500-2000. IHC 1:50-200. IF 1:50-200.
交叉反应 Human Mouse Rat
蛋白分子量 71kDa
Gene ID 5830
保存 Store at -20°C. Avoid freeze / thaw cycles.
储存液 Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
纯化方法 Affinity purification
亚型 IgG
免疫原 Recombinant protein of human PEX5
性状 液体
Public Immunogen Range Recombinant protein of human PEX5
Subcellular Locations Cytoplasm Peripheral membrane protein Peroxisome membrane
Swiss Prot P50542
克隆类型 Polyclonal Antibody
背景资料 The product of this gene 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). Alternatively spliced transcript variants encoding different isoforms have been identified.