CLC7 Peptide | CLCN7 peptide

CLC7, Human (Clcn7, Chloride Channel) Control Peptide

Cat. Num. AAA659902

• Gene Names: CLCN7; CLC7; CLC-7; OPTA2; OPTB4; PPP1R63
• Applications: ELISA, Western Blot
• Purity: Highly Purified; Highly purified
• Synonyms: CLC7; Human (Clcn7; Chloride Channel) Control Peptide; CLCN7 peptide

• Host: Human
• Specificity: 93% conserved in mouse/rat CLC-7. No significant sequence homology is detected with other CLCs or other proteins
• Purity/Purification: Highly Purified; Highly purified
• Form/Format: Supplied as a liquid in PBS, pH 7.2

• Applicable Applications for CLCN7 peptide: ELISA (EL/EIA), Western Blot (WB)
• Preparation and Storage: -20 degree C

Related Product Information for CLCN7 peptide

A 15aa Peptide sequence near the N-terminus of human CLC-7.

Chloride is a critical component of all living cells. Voltage-gated chloride channels regulate cellular traffic of chloride ion. The chloride channels (CIC or CLC) performs several functions including the regulation of cell volume, membrane potential stabilization, signal transduction, and transepithelial transport. Mutations in CIC genes have been linked with several human diseases including myotonias (Thomsen's disease), cystic fibrosis, Bartters syndrome type III, Dent's disease, and X-linked recessive nephrolithiasis. In mammals, CLC proteins form a superfamily of at least 9 different genes (CLC1-7 also known as CLCN1-7 and CLK1-2 or CLCKa and CLCKb). Additional forms of these proteins are obtained by alternative splicing. All CLC proteins (~700-1000 aa) are predicted to contain 10 (possibly 12) transmembrane domains. Except CLC-1 and CLC-K1/K2 that are specific for kidney, most other CLC are widely distributed in various tissues.
Rat CLC-7 is 802 aa membrane protein (mouse 803 aa, human 805 aa) (1). CLC-7 has a calculated MW of approx. 90kD. CLC-7 is ~45% identical with CLC-6. CLC-7 is expressed in brain, tested, muscle and kidney (1.

Product Categories/Family for CLCN7 peptide

Molecular Biology; MB-Ion Channel

NCBI and Uniprot Product Information

• NCBI GI #: 15215300
• NCBI GeneID: 1186
• UniProt Accession #: P51798; Q9NYX5; A6NEJ7; A8K5T9; A8K7X1; B3KPN3; E9PDB9
• Molecular Weight: 88,679 Da
• NCBI Official Full Name: Chloride channel 7
• 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

Function: Slowly voltage-gated channel mediating the exchange of chloride ions against protons. Functions as antiporter and contributes to the acidification of the lysosome lumen. Ref.10 Ref.13

Subunit structure: Chloride channel 7 are heteromers of alpha (CLCN7) and beta (OSTM1) subunits. Ref.13

Subcellular location: Lysosome membrane; Multi-pass membrane protein Ref.9 Ref.10 Ref.13.

Tissue specificity: Brain, testis, muscle and kidney.

Involvement in disease: Osteopetrosis, autosomal recessive 4 (OPTB4) [MIM:611490]: A rare genetic disease characterized by abnormally dense bone, due to defective resorption of immature bone. Osteopetrosis 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. Recessive osteopetrosis commonly manifests in early infancy with macrocephaly, feeding difficulties, evolving blindness and deafness, bone marrow failure, severe anemia, and hepatosplenomegaly. Deafness and blindness are generally thought to represent effects of pressure on nerves.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.15 Ref.16 Ref.17 Ref.18 Ref.19Osteopetrosis, autosomal dominant 2 (OPTA2) [MIM:166600]: A rare genetic disease characterized by abnormally dense bone, due to defective resorption of immature bone. Osteopetrosis 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.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.16 Ref.17 Ref.19

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

By similarity.

Sequence similarities: Belongs to the chloride channel (TC 2.A.49) family. ClC-7/CLCN7 subfamily. [View classification]Contains 2 CBS domains.