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折扣/货期| 货号：GTX45112 | 规格：50μg | 目录价：￥6300 |
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:
NPHP1 antibody, Internal
别名:
nephrocystin 1 , JBTS4 , NPH1 , SLSN1
反应种属:
Human
宿主来源:
Rabbit
实验应用:
WB
同种型:
IgG
免疫原:
A synthetic peptide corresponding to an Internal region of Human NPHP1
克隆性:
Polyclonal
克隆号:
纯化方式:
Affinity Purified
偶联:
Unconjugated
产品浓度:
0.5-1 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:
83
产品形式:
Liquid
存储溶液:
PBS, 2% Sucrose, 0.09% Sodium azide.
生产商:
GeneTex
功能与背景:
This gene encodes a protein with src homology domain 3 (SH3) patterns. This protein interacts with Crk-associated substrate, and it appears to function in the control of cell division, as well as in cell-cell and cell-matrix adhesion signaling, likely as part of a multifunctional complex localized in actin- and microtubule-based structures. Mutations in this gene cause familial juvenile nephronophthisis type 1, a kidney disorder involving both tubules and glomeruli. Defects in this gene are also associated with Senior-Loken syndrome type 1, also referred to as juvenile nephronophthisis with Leber amaurosis, which is characterized by kidney and eye disease, and with Joubert syndrome type 4, which is characterized by cerebellar ataxia, oculomotor apraxia, psychomotor delay and neonatal breathing abnormalities, sometimes including retinal dystrophy and renal disease. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]
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