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Merck
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主要文件

SAB5702103

Sigma-Aldrich

Anti-PEX19 Antibody, clone 9O7Q9, Rabbit Monoclonal

别名:

PEX19, D1S2223E, HK33, PBD12A, PMP1, PMPI, PXF, PXMP1, peroxisomal biogenesis factor 19

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About This Item

分類程式碼代碼:
12352203
NACRES:
NA.46

生物源

rabbit

品質等級

材料

colorless

無性繁殖

9O7Q9, monoclonal

形狀

liquid

分子量

35 kDa

物種活性

rat, human

濃度

0.4 mg/mL

技術

western blot: 1:500 - 1:2000

顏色

colorless

同型

IgG

免疫原序列

MAAAEEGCSVGAEADRELEELLESALDDFDKAKPSPAPPSTTTAPDASGPQKRSPGDTAKDALFASQEKFFQELFDSELASQATAEFEKAMKELAEEEPH

UniProt登錄號

運輸包裝

wet ice

儲存溫度

−20°C

目標翻譯後修改

unmodified

基因資訊

human ... PEX19(5824)

一般說明

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.

應用

WB

儲存類別代碼

10 - Combustible liquids

水污染物質分類(WGK)

WGK 1


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