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折扣/货期| 货号：GTX628892-S | 规格：25μl | 目录价：￥1700 |
| 货号：GTX628892 | 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:
PEX19 antibody [GT533]
别名:
peroxisomal biogenesis factor 19 , D1S2223E , HK33 , PBD12A , PMP1 , PMPI , PXF , PXMP1
反应种属:
Human, Rat
宿主来源:
Mouse
实验应用:
FCM, ICC/IF, WB
同种型:
IgG2b
免疫原:
Recombinant protein encompassing a sequence within the center region of human PEX19. The exact sequence is proprietary.
克隆性:
Monoclonal
克隆号:
GT533
纯化方式:
Affinity purified by Protein G.
偶联:
Unconjugated
产品浓度:
1.17 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:
33
产品形式:
Liquid
存储溶液:
PBS, no preservatives.
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. 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 Zellweger syndrome (ZWS). [provided by RefSeq]
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