生物来源
rabbit
质量水平
物料
colorless
克隆
9O7Q9, monoclonal
形式
liquid
分子量
35 kDa
种属反应性
rat, human
浓度
0.4 mg/mL
技术
western blot: 1:500 - 1:2000
颜色
colorless
同位素/亚型
IgG
免疫原序列
MAAAEEGCSVGAEADRELEELLESALDDFDKAKPSPAPPSTTTAPDASGPQKRSPGDTAKDALFASQEKFFQELFDSELASQATAEFEKAMKELAEEEPH
UniProt登记号
运输
wet ice
储存温度
−20°C
靶向翻译后修饰
unmodified
基因信息
human ... PEX19(5824)
一般描述
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.
免疫原
A synthetic peptide corresponding to a sequence within amino acids 1-100 of human PEX19 (P40855).
PBS with 0.02% sodium azide,0.05% BSA,50% glycerol,pH7.3.
应用
WB
WGK
WGK 1
法规信息
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