Testing Data
Rabbit Connexin 43 Antibody | anti-Connexin 43 antibody
Rabbit anti Connexin 43 antibody
ELISA: 0.01-0.1 ug/ml
Immunoprecipitation: 2-5 ug/ml
Testing Data
Testing Data
NCBI and Uniprot Product Information
NCBI Description
This gene is a member of the connexin gene family. The encoded protein is a component of gap junctions, which are composed of arrays of intercellular channels that provide a route for the diffusion of low molecular weight materials from cell to cell. The encoded protein is the major protein of gap junctions in the heart that are thought to have a crucial role in the synchronized contraction of the heart and in embryonic development. A related intronless pseudogene has been mapped to chromosome 5. Mutations in this gene have been associated with oculodentodigital dysplasia and heart malformations. [provided by RefSeq]
Uniprot Description
Function: One gap junction consists of a cluster of closely packed pairs of transmembrane channels, the connexons, through which materials of low MW diffuse from one cell to a neighboring cell. May play a critical role in the physiology of hearing by participating in the recycling of potassium to the cochlear endolymph.
Subunit structure: A connexon is composed of a hexamer of connexins. Interacts with SGSM3. Interacts with KIAA1432/CIP150. Interacts with CNST. Ref.16
Subcellular location: Cell membrane; Multi-pass membrane protein. Cell junction › gap junction.
Tissue specificity: Expressed in the heart and fetal cochlea. Ref.15
Involvement in disease: Defects in GJA1 are the cause of autosomal dominant oculodentodigital dysplasia (ODDD) [
MIM:164200]; also known as oculodentoosseous dysplasia. ODDD is a highly penetrant syndrome presenting with craniofacial (ocular, nasal, dental) and limb dysmorphisms, spastic paraplegia, and neurodegeneration. Craniofacial anomalies tipically include a thin nose with hypoplastic alae nasi, small anteverted nares, prominent columnella, and microcephaly. Brittle nails and hair abnormalities of hypotrichosis and slow growth are present. Ocular defects include microphthalmia, microcornea, cataracts, glaucoma, and optic atrophy. Syndactyly type 3 and conductive deafness can occur in some cases. Cardiac abnormalities are observed in rare instances. Ref.17 Ref.19 Ref.22 Ref.24 Ref.25 Ref.26 Ref.27 Ref.30 Ref.31 Ref.32 Ref.33 Ref.34 Ref.35Defects in GJA1 are the cause of autosomal recessive oculodentodigital dysplasia (ODDD autosomal recessive) [
MIM:257850].Defects in GJA1 may be the cause of syndactyly type 3 (SDTY3) [
MIM:186100]. Syndactyly is an autosomal dominant trait and is the most common congenital anomaly of the hand or foot. It is marked by persistence of the webbing between adjacent digits, so they are more or less completely attached. In this type there is usually complete and bilateral syndactyly between the fourth and fifth fingers. Usually it is soft tissue syndactyly but occasionally the distal phalanges are fused. The fifth finger is short with absent or rudimentary middle phalanx. The feet are not affected. Ref.24Defects in GJA1 are a cause of hypoplastic left heart syndrome (HLHS) [
MIM:241550]. HLHS refers to the abnormal development of the left-sided cardiac structures, resulting in obstruction to blood flow from the left ventricular outflow tract. In addition, the syndrome includes underdevelopment of the left ventricle, aorta, and aortic arch, as well as mitral atresia or stenosis. Ref.21Defects in GJA1 are a cause of Hallermann-Streiff syndrome (HSS) [
MIM:234100]. HSS is a disorder characterized by a typical skull shape (brachycephaly with frontal bossing), hypotrichosis, microphthalmia, cataracts, beaked nose, micrognathia, skin atrophy, dental anomalies and proportionate short stature. Mental retardation is present in a minority of cases. Ref.23
Sequence similarities: Belongs to the connexin family. Alpha-type (group II) subfamily.
Caution: Ref.20 reported a mutation Pro-364 linked to congenital heart diseases. Ref.12 later shown that it is an artifact.Ref.15 reported 2 mutations (Phe-11 and Ala-24) linked to non-syndromic autosomal recessive deafness (DFNBG). These mutations have subsequently been shown (Ref.19) to involve the pseudogene of connexin-43 located on chromosome 5.
Research Articles on Connexin 43
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Product Notes
The Connexin 43 gja1 (Catalog #AAA462036) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Rabbit anti Connexin 43 antibody reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's Connexin 43 can be used in a range of immunoassay formats including, but not limited to, ELISA, Western Blot, Immunohistochemistry. Western Blot: 0.1-1 ug/ml ELISA: 0.01-0.1 ug/ml Immunoprecipitation: 2-5 ug/ml. Researchers should empirically determine the suitability of the Connexin 43 gja1 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 "Connexin 43, 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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