Anti-PEX19 Polyclonal Antibody
品牌:solarbio | 货号:K004023P
英文名称 |
Anti-PEX19 Polyclonal Antibody |
宿主 |
Rabbit |
别名 |
D1S2223E;HK33;PBD12A;PMP1;PMPI;PXF;PXMP1 |
应用 |
WB IHC |
稀释比例 |
WB 1:500-2000. IHC 1:50-200. |
交叉反应 |
Human Mouse |
蛋白分子量 |
33kDa |
Gene ID |
5824 |
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 PEX19 |
性状 |
液体 |
Public Immunogen Range |
Recombinant protein of human PEX19 |
Subcellular Locations |
Cytoplasm Cytoplasmic side Lipid-anchor Peroxisome membrane |
Swiss Prot |
P40855 |
克隆类型 |
Polyclonal Antibody |
背景资料 |
This gene is necessary for early peroxisomal biogenesis. It acts both as a cytosolic chaperone and as an import receptor for peroxisomal membrane proteins (PMPs). 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. These disorders have at least 14 complementation groups, with more than one phenotype being observed for some 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 Zellweger syndrome (ZWS), as well as peroxisome biogenesis disorder complementation group 14 (PBD-CG14), which is also known as PBD-CGJ. Alternative splicing results in multiple transcript variants. |