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折扣/货期| 货号：GTX32780 | 规格：100μl | 目录价：￥5000 |
产品详情
* 以下信息仅供参考，详情请以原厂网站为准
产品名称:
PEX19 antibody
别名:
peroxisomal biogenesis factor 19 , D1S2223E , HK33 , PBD12A , PMP1 , PMPI , PXF , PXMP1
反应种属:
Human, Mouse
宿主来源:
Rabbit
实验应用:
IHC-P, WB
同种型:
IgG
免疫原:
Recombinant fusion protein containing a sequence corresponding to amino acids 1-299 of human PEX19 (NP_002848.1).
克隆性:
Polyclonal
克隆号:
纯化方式:
Purified by affinity chromatography
偶联:
Unconjugated
产品浓度:
Batch dependent (Please refer to the vial label for the specific concentration.)
保存温度:
Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4ºC. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.
运输温度:
4°C
预期分子量:
33
产品形式:
Liquid
存储溶液:
PBS, 50% Glycerol, 0.02% Sodium azide.
生产商:
GeneTex
功能与背景:
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. [provided by RefSeq, Aug 2010]
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