- 产品信息
商品描述
靶点-Target
NPHP1
反应性
Human,Mouse
应用
WB,ELISA
宿主物种-Host Species
Rabbit
同种型-Isotype
IgG
修饰-Modification
Unmodified
MW(Observed)
80kD
推荐稀释比
WB 1:500-2000;ELISA 1:5000-20000
组成
PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
纯化工艺
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
储存
-15°C to -25°C/1 year(Do not lower than -25°C)
浓度
1 mg/ml
克隆性
Polyclonal
免疫原
Synthesized peptide derived from human protein . at AA range: 510-590
特异性
NPHP1 Polyclonal Antibody detects endogenous levels of protein.
基因名称
NPHP1 NPH1
蛋白名称
Nephrocystin-1 (Juvenile nephronophthisis 1 protein)
Organism-1
Human
基因ID-1
4867
SwissProt-1
O15259
Organism-2
Mouse
SwissProt-2
Q9QY53
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 diffe
细胞定位
Cell junction . Cell junction, adherens junction . Cell projection, cilium . Cytoplasm, cytoskeleton, cilium axoneme . Cell junction, tight junction. In the retinal photoreceptor cell layer, localizes at the connecting cilium (By similarity). Colocalizes with E-cadherin and BCAR1 at or near the cell-cell adherens junctions (By similarity). Localized to respiratory cilia axoneme (PubMed:16308564, PubMed:16885411). Localized to the transition zone of respiratory cilia (PubMed:16885411). Localized to the transition zone of photoreceptor-connecting cilia and renal monocilia (By similarity). In cultured renal cells, it localizes diffusely in the cytoplasm but, as cells approach confluence, it accumulates at basolateral tight junctions (By similarity). .





