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. |