BD-PT0961

CLC-7 Rabbit Polyclonal Antibody

CLCN7; H(+)/Cl(-) exchange transporter 7; Chloride channel 7 alpha subunit; Chloride channel protein 7; ClC-7

常规抗体

CLCN7

H(+)/Cl(-) exchange transporter 7

WB

Human,Mouse,Rat

1 mg/ml

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.

1186

http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=1186

P51798

http://www.uniprot.org/uniprotkb/P51798/entry

26373

http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=26373

O70496

http://www.uniprot.org/uniprot/O70496

29233

http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=29233

P51799

http://www.uniprot.org/uniprot/P51799

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

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

WB 1:500 - 1:2000. ELISA: 1:40000. Not yet tested in other applications.

90kD

-20°C/1 year

Polyclonal, Rabbit,IgG

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],

Brain and kidney.

Lysosome membrane ; Multi-pass membrane protein .

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 ions against protons. Functions as antiporter and contributes to the acidification of the lysosome lumen.,miscellaneous:The CLC channel family contains both chloride channels and proton-coupled anion transporters that exchange chloride or another anion for protons. The presence of conserved gating glutamate residues is typical for family members that function as antiporters.,similarity:Belongs to the chloride channel (TC 2.A.49) family.,similarity:Contains 2 CBS domains.,tissue specificity:Brain, testis, muscle and kidney.,

现货

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