CLCN7, Polyclonal Antibody

CLC-7; CLC7; FLJ26686; FLJ39644; FLJ46423; OPTA2; OPTB4

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

Antigen affinity purification

Store at -20 degree C. Avoid freeze / thaw cycles

Manufactured in an ISO 9001:2015 Certified Laboratory.

Small volumes of anti-CLCN7 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

ELISA (EIA)

86,026 Da

chloride channel, voltage-sensitive 7

H(+)/Cl(-) exchange transporter 7; H(+)/Cl(-) exchange transporter 7; chloride channel 7 alpha subunit; chloride channel protein 7; protein phosphatase 1, regulatory subunit 63

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

ClC-7  [Similar Products]

CLCN7_HUMAN

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 *****hood. [provided by RefSeq, Jul 2008]

CLCN7: Slowly voltage-gated channel mediating the exchange of chloride ions against protons. Functions as antiporter and contributes to the acidification of the lysosome lumen. Defects in CLCN7 are the cause of osteopetrosis autosomal recessive type 4 (OPTB4); also known as 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 *****hood. Defects in CLCN7 are the cause of osteopetrosis autosomal dominant type 2 (OPTA2); also known as autosomal dominant Albers-Schonberg disease or marble disease autosomal dominant. 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 *****hood. OPTA2 is the most common form of osteopetrosis, occurring in adolescence or *****hood. It is characterized by sclerosis, predominantly involving the spine, the pelvis and the skull base. Belongs to the chloride channel (TC 2.A.49) family. ClC-7/CLCN7 subfamily.

Protein type: Transporter, ion channel; Membrane protein, integral; Transporter; Membrane protein, multi-pass; Channel, chloride

Chromosomal Location of Human Ortholog: 16p13

Cellular Component: membrane; lysosomal membrane; integral to membrane; cytoplasmic vesicle

Molecular Function: chloride channel activity; voltage-gated chloride channel activity; ATP binding; antiporter activity

Biological Process: transport; transmembrane transport; response to pH

Disease: Osteopetrosis, Autosomal Dominant 2; Osteopetrosis, Autosomal Recessive 4

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