Rabbit anti-Human CLCN7 Polyclonal Antibody | anti-CLCN7 antibody

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

Cat. Num. AAA6008945

• Gene Names: CLCN7; CLC7; CLC-7; OPTA2; OPTB4; PPP1R63
• Reactivity: Human
• Applications: ELISA, Western Blot, Immunohistochemistry, Immunofluorescence
• Purity: Affinity Purified; Purified by Protein A affinity chromatography.
• Synonyms: CLCN7; Polyclonal Antibody; CT (CLCN7; H(+)/Cl(-) exchange transporter 7; Chloride channel 7 alpha subunit; Chloride channel protein 7); Anti -CLCN7; anti-CLCN7 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Human
• Purity/Purification: Affinity Purified; Purified by Protein A affinity chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.

• Applicable Applications for anti-CLCN7 antibody: ELISA (EL/EIA), Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)
• Application Notes: Suitable for use in Western Blot, Immunohistochemistry, Immunofluorescence, ELISA; Dilution: ELISA: 1:1,000; Western Blot: 1:100-500; Immunohistochemistry: 1:10-50; Immunofluorescence: 1:10-50
• Immunogen: CLCN7 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 699-728 amino acids from the C-terminal region of human CLCN7.
• Preparation and Storage: May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(CLCN7 Antibody (C-term) western blot analysis in CEM cell line lysates (35ug/lane).This demonstrates the CLCN7 antibody detected the CLCN7 protein (arrow).)

Immunohistochemistry (IHC)

(CLCN7 Antibody (C-term) immunohistochemistry analysis in formalin fixed and paraffin embedded human kidney tissue followed by peroxidase conjugation of the secondary antibody and DAB staining.This data demonstrates the use of CLCN7 Antibody (C-term) for immunohistochemistry. Clinical relevance has not been evaluated.)

Immunofluorescence (IF)

(Confocal immunofluorescent analysis of CLCN7 Antibody (C-term) with WiDr cell followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green). DAPI was used to stain the cell nuclear (blue).)

Related Product Information for anti-CLCN7 antibody

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.

Product Categories/Family for anti-CLCN7 antibody

Antibodies; Abs to Transport Proteins

NCBI and Uniprot Product Information

• NCBI GI #: 13544046
• NCBI GeneID: 1186
• UniProt Accession #: P51798; Q9NYX5; A6NEJ7; A8K5T9; A8K7X1; B3KPN3; E9PDB9
• Molecular Weight: 88,679 Da
• NCBI Official Full Name: CLCN7 protein, partial
• NCBI Official Synonym Full Names: chloride channel, voltage-sensitive 7
• NCBI Official Symbol: CLCN7
• NCBI Official Synonym Symbols: CLC7; CLC-7; OPTA2; OPTB4; PPP1R63
• NCBI Protein Information: H(+)/Cl(-) exchange transporter 7; H(+)/Cl(-) exchange transporter 7; chloride channel protein 7; chloride channel 7 alpha subunit; protein phosphatase 1, regulatory subunit 63
• UniProt Protein Name: H(+)/Cl(-) exchange transporter 7
• Protein Family: H(+)/Cl(-) exchange transporter
• UniProt Gene Name: CLCN7
• UniProt Synonym Gene Names: ClC-7
• UniProt Entry Name: CLCN7_HUMAN

NCBI Description

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]

Uniprot Description

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 adulthood. 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 adulthood. 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. Belongs to the chloride channel (TC 2.A.49) family. ClC-7/CLCN7 subfamily.

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

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