Rabbit anti-Human CLC7 Polyclonal Antibody | anti-CLCN7 antibody
CLC7 (Clcn7, Chloride Channel)
Purified by immunoaffinity chromatography.
Purified by immunoaffinity chromatography.
Dilution: Western Blot: 1-10ug/ml (ECL.)
ELISA: 0.5-1ug/ml Control peptide can be used to coat ELISA plates at 1ug/ml.
NCBI and Uniprot Product Information
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]
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: Membrane protein, multi-pass; Transporter; Transporter, ion channel; Membrane protein, integral; 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
Research Articles on CLCN7
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Product Notes
The CLCN7 clcn7 (Catalog #AAA611101) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The CLC7 (Clcn7, Chloride Channel) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's CLC7 can be used in a range of immunoassay formats including, but not limited to, ELISA (EL/EIA), Western Blot (WB). Suitable for use in ELISA, Western Blot. Dilution: Western Blot: 1-10ug/ml (ECL.) ELISA: 0.5-1ug/ml Control peptide can be used to coat ELISA plates at 1ug/ml. Researchers should empirically determine the suitability of the CLCN7 clcn7 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "CLC7, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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