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Key Documents

SAB4200730

Sigma-Aldrich

Anti-Connexin 43 Antibody

mouse monoclonal, CXN-6

Synonym(s):

Anti-AVSD3, Anti-CMDR, Anti-CX43, Anti-EKVP, Anti-EKVP3, Anti-GJAL, Anti-HLHS1, Anti-HSS, Anti-ODDD, Anti-PPKCA

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About This Item

UNSPSC Code:
12352203
NACRES:
NA.41

product name

Anti-Connexin 43 antibody, Mouse monoclonal, clone CXN-6, hybridoma cell culture supernatant

biological source

mouse

Quality Level

antibody form

culture supernatant

antibody product type

primary antibodies

clone

CXN-6, monoclonal

form

buffered aqueous solution

species reactivity

chicken, rat, feline, bovine, human, porcine, mouse

packaging

antibody small pack of 25 μL

concentration

~1.0 mg/mL

technique(s)

immunoblotting: 1:5,000-1:7,500 using mouse myoblast C2C12 cell line extract
immunofluorescence: 1:500-1:1,000 using mouse myoblast C2C12 cell line
immunohistochemistry: 1:200-1:500 using heat-retrieved formalin-fixed, paraffin-embedded mouse heart sections

isotype

IgM

UniProt accession no.

shipped in

dry ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

bovine ... Gja1(281193)
cat ... Gja1(101100211)
chicken ... Gja1(395278)
human ... GJA1(2697)
mouse ... Gja1(14609)
rat ... Gja1(24392)

General description

Connexin 43 (Cx43) is also known as gap junction alpha-1 protein (GJA1). It is mapped to human chromosome 6q22.31. Cx43 is membrane protein with four transmembrane spanning regions, which comprise the gap junction channel. The loop connecting the transmembrane regions play a key role in channel docking. The N- and C-terminal amino acids are exposed to cytoplasm.

Immunogen

synthetic peptide from the C-terminal region of Connexin 43 protein, conjugated to KLH

Biochem/physiol Actions

Connexin 43 (Cx43) function is controlled by phosphorylation, SUMOylation and ubiquitination. The C-terminal tail of Cx43 modulates cytoskeletal dynamics and cell migration properties. Mutation in Cx43 leads to dysfunction of channel regulation domain and is associated with pathogenesis of hypoplastic left heart syndrome (HLHS). Cx43 dysregulation in testis contributes to infertility and testicular tumor. A gene mutation in the Cx43 results in abnormal development of eyes and teeth in oculodentodigital dysplasia (ODDD) , craniometaphyseal dysplasia and sudden infant death syndrome. Mutations in the GJA1 results in abnormal facial and skull development in hallermann-streiff syndrome (HSS). Missense mutations in GJA1 gene is also implicated in skin disorder called the, erythrokeratodermia variabilis et progressiva (EKVP)

Physical form

The product is supplied as a culture supernatant solution containing 15 mM sodium azide as a preservative. The product contains bovine serum albumin and a human-derived protein.

Disclaimer

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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Storage Class Code

10 - Combustible liquids

WGK

WGK 1

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable


Certificates of Analysis (COA)

Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.

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Dominant de novo mutations in GJA1 cause erythrokeratodermia variabilis et progressiva, without features of oculodentodigital dysplasia
Boyden LM, et al.
The Journal of Investigative Dermatology, 135(6), 1540-1547 (2015)
A case of oculodentodigital dysplasia syndrome with novel GJA1 gene mutation
Himi M, et al.
Japanese Journal of Ophthalmology, 53(5), 541-541 (2009)
A homozygous GJA1 gene mutation causes a Hallermann-Streiff/ODDD spectrum phenotype
Pizzuti A, et al.
Febs Letters, 23(3), 286-286 (2004)
Identification of connexin43 (alpha1) gap junction gene mutations in patients with hypoplastic left heart syndrome by denaturing gradient gel electrophoresis (DGGE)
Dasgupta C, et al.
Mutation Research. Fundamental and Molecular Mechanisms of Mutagenesis, 479(1), 173-186 (2001)
Structural organization of gap junction channels
Sosinsky GE and Nicholson BJ
Biochimica et Biophysica Acta - Biomembranes, 1711(2), 99-125 (2005)

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