靶标:
PEX19
产品别名:
D1S2223E; HK33; PBD12A; PMP1; PMPI; PXF; PXMP1; PEX19; peroxisomal biogenesis factor 19; peroxisomal biogenesis factor 19; peroxisomal biogenesis factor 19; 33 kDa housekeeping protein; housekeeping gene, 33kD; peroxin-19; peroxisomal farnesylated protein; 过氧化物酶体生物合成因子19;
背景信息:
peroxisomal biogenesis factor 19(PEX19) Homo sapiens 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
宿主:Rbt
类型:Pab
同种型:IgG
应用:WB
纯化方式:亲和纯化
偶联物:Unconjugated
性状:液体
存储溶液:参阅说明书
浓度:Batch dependent (Please refer to the vial label for the specific concentration.)
稀释比例: Optimal dilutions/concentrations should be determined by the end user
储存:经常使用则4°C保存。-20°C保存不超过两年。避免反复冻融。
注意事项:仅供实验室使用。不适用于人类或动物的任何临床,治疗或诊断用途。不适合动物或人类食用。