免疫原
Synthesized peptide derived from part region of human protein
特异性
CLCN5 Polyclonal Antibody detects endogenous levels of protein.
来源
Polyclonal, Rabbit,IgG
组成（Formulation）
Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.
稀释比例
WB 1:500-2000 ELISA 1:5000-20000
纯化工艺（Immunogen）
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
背景（Background）
chloride voltage-gated channel 5(CLCN5) Homo sapiens This gene encodes a member of the ClC family of chloride ion channels and ion transporters. The encoded protein is primarily localized to endosomal membranes and may function to facilitate albumin uptake by the renal proximal tubule. Mutations in this gene have been found in Dent disease and renal tubular disorders complicated by nephrolithiasis. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Jan 2013],
功能
disease:Defects in CLCN5 are a cause of hypophosphatemic rickets X-linked recessive (XLRH) [MIM:300554]. XLRH is a renal disease belonging to the 'Dent disease complex', a group of disorders characterized by proximal renal tubular defect, hypercalciuria, nephrocalcinosis, and renal insufficiency. The spectrum of phenotypic features is remarkably similar in the various disorders, except for differences in the severity of bone deformities and renal impairment. XLRH patients present with rickets or osteomalacia, hypophosphatemia due to decreased renal tubular phosphate reabsorption, hypercalciuria, and low molecualr weight proteinuria. Patients develop nephrocalcinosis with progressive renal failure in adulthood. Female carriers may have asymptomatic hypercalciuria or hypophosphatemia only.,disease:Defects in CLCN5 are the cause of low molecular weight proteinuria with hypercalciuria and ne
基因名称（Gene Name）
CLCN5 CLCK2
蛋白名称
H(+)/Cl(-) exchange transporter 5 (Chloride channel protein 5) (ClC-5) (Chloride transporter ClC-5)
Fields
>>Neutrophil extracellular trap formation
细胞定位
Golgi apparatus membrane ; Multi-pass membrane protein . Endosome membrane ; Multi-pass membrane protein . Cell membrane ; Multi-pass membrane protein .
组织表达
Kidney. Moderately expressed in aortic vascular smooth muscle and endothelial cells, and at a slightly higher level in the coronary vascular smooth muscle.
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