antibody

CLCN7 Antibody

MBS9429658

0.1 mL

255 USD

polyclonal

rabbit

nonconjugated

western blot, immunohistochemistry

Antibody

CLCN7 Antibody

[CLCN7]

[CLC7; CLC-7; OPTA2; OPTB4; PPP1R63]

[Homo sapiens chloride channel 7, mRNA; H(+)/Cl(-) exchange transporter 7; H(+)/Cl(-) exchange transporter 7; chloride voltage-gated channel 7; Chloride channel 7 alpha subunit; Chloride channel protein 7; ClC-7]

[CLCN7]

[CLCN7; CLCN7; CLC7; CLC-7; OPTA2; OPTB4; PPP1R63; ClC-7]

Polyclonal

IgG

Rabbit

Human

3760

The antibody detects endogenous levels of total CLCN7 protein.

Antigen affinity purification

Rabbit IgG in pH 7.4 PBS 0.05% NaN3, 40% Glycerol.

0.7mg/ml

Store at -20°C.

Western Blot (WB), Immunohistochemistry (IHC)

WB: 1:200-1:1000

SDS-Page

Immunogen: Synthetic protein corresponding to residues near the C terminal of human CLCN7. Immunogen Type: Peptide. Calculated MW: 89 kDa

Total protein Ab

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.

39644913

BC012737

P51798

Ion Channel Transport Pathway (1269950); Stimuli-sensing Channels Pathway (1269953); Transmembrane Transport Of Small Molecules Pathway (1269903)

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]

Slowly voltage-gated channel mediating the exchange of chloride ions against protons. Functions as antiporter and contributes to the acidification of the lysosome lumen.

0.1 mL

255 USD