Product Description

CLC-7 Rabbit Polyclonal Antibody

Sizes: 50μL, 100μL

Catalogue Numbers: ES1993-50, ES1993-100

Lead times: 1-2 weeks, if manufacturer has product in stock

Manufacturer/Ship Location: China

Background: chloride voltage-gated channel 7(CLCN7) Homo sapiens The product of this gene belongs to the CLC chloride channel family of proteins. Chloride channels play important roles in the plasma membrane and in intracellular organelles. This gene encodes chloride channel 7. Defects in this gene are the cause of osteopetrosis autosomal recessive type 4 (OPTB4), also called infantile malignant osteopetrosis type 2 as well as the cause of autosomal dominant osteopetrosis type 2 (OPTA2), also called autosomal dominant Albers-Schonberg disease or marble disease autosoml dominant. Osteopetrosis is a rare genetic disease characterized by abnormally dense bone, due to defective resorption of immature bone. OPTA2 is the most common form of osteopetrosis, occurring in adolescence or adulthood. [provided by RefSeq, Jul 2008],

Reactivity: Human, Mouse, Rat

Host: Rabbit

Applications: WB, ELISA

Alternate Names: CLCN7, H(+)/Cl(-) exchange transporter 7, Chloride channel 7 alpha subunit, Chloride channel protein 7, ClC-7

Clonality: Polyclonal

Immunogen: The antiserum was produced against synthesized peptide derived from human CLCN7. AA range:10-59

Isotype: IgG

Target: CLC-7

Specificity: CLC-7 Polyclonal Antibody detects endogenous levels of CLC-7 protein.

Dilution: Western Blot: 1/500 - 1/2000. ELISA: 1/40000. Not yet tested in other applications.

Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration: 1 mg/ml,

Formlation: Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Expression: Brain and kidney,

Observed Band (KD): 90kD

Function: Disease: Defects in CLCN7 are a cause of autosomal dominant osteopetrosis type 2 (OPTA2) [MIM: 166600], also called autosomal dominant Albers-Schonberg disease or marble disease autosoml dominant. OPTA2 is the most common form of osteopetrosis, occurring in adolescence or adulthood. It is characterized by sclerosis, predominantly involving the spine, the pelvis, and the skull base
Disease: Defects in CLCN7 are the cause of osteopetrosis autosomal recessive type 4 (OPTB4) [MIM: 611490], also called infantile malignant osteopetrosis type 2. Osteopetrosis is a rare genetic disease characterized by abnormally dense bone, due to defective resorption of immature bone. The disorder occurs in two forms: a severe autosomal recessive form occurring in utero, infancy, or childhood, and a benign autosomal dominant form occurring in adolescence or adulthood
Function: Mediates the exchange of chloride io

Subcellular Location: Lysosome membrane, Multi-pass membrane protein

Storage and Stability: -20°C/1 year

Gene Name: CLCN7

Protein Name: H(+)/Cl(-) exchange transporter 7

Mouse Gene ID: 26373

Mouse SwissProt Number: O70496

Rat Gene ID: 29233

Rat SwissProt Number: P51799

Research Use Only