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折扣/货期| 货号：GTX109798-S | 规格：25μl | 目录价：￥1700 |
| 货号：GTX109798 | 规格：100μl | 目录价：￥4000 |
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:
PEX5 antibody
别名:
peroxisomal biogenesis factor 5 , PBD2A , PBD2B , PTS1-BP , PTS1R , PXR1 , RCDP5
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 protein encompassing a sequence within the center region of human PEX5. The exact sequence is proprietary.
克隆性:
Polyclonal
克隆号:
纯化方式:
Purified by antigen-affinity chromatography.
偶联:
Unconjugated
产品浓度:
1 mg/ml (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:
71
产品形式:
Liquid
存储溶液:
0.1M Tris, 0.1M Glycine, 10% Glycerol, 0.01% Thimerosal.
生产商:
GeneTex
功能与背景:
The product of this gene binds to the C-terminal PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import. 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. The peroxisomal biogenesis disorders are a heterogeneous group with at least 14 complementation groups and with more than 1 phenotype being observed in cases falling into particular 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 neonatal adrenoleukodystrophy (NALD), a cause of Zellweger syndrome (ZWS) as well as may be a cause of infantile Refsum disease (IRD). Alternatively spliced transcript variants encoding different isoforms have been identified. [provided by RefSeq]
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