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Merck

SAB4200730

Sigma-Aldrich

Anti-Connexin 43 antibody, Mouse monoclonal

clone CXN-6, hybridoma cell culture supernatant

Synonim(y):

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

Kod UNSPSC:
12352203
NACRES:
NA.41

pochodzenie biologiczne

mouse

Poziom jakości

forma przeciwciała

culture supernatant

rodzaj przeciwciała

primary antibodies

klon

CXN-6, monoclonal

Postać

buffered aqueous solution

reaktywność gatunkowa

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

opakowanie

antibody small pack of 25 μL

stężenie

~1.0 mg/mL

metody

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

izotyp

IgM

numer dostępu UniProt

Warunki transportu

dry ice

temp. przechowywania

−20°C

docelowa modyfikacja potranslacyjna

unmodified

informacje o genach

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

Opis ogólny

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

Działania biochem./fizjol.

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)

Postać fizyczna

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.

Oświadczenie o zrzeczeniu się odpowiedzialności

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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Kod klasy składowania

10 - Combustible liquids

Klasa zagrożenia wodnego (WGK)

WGK 1

Temperatura zapłonu (°F)

Not applicable

Temperatura zapłonu (°C)

Not applicable


Certyfikaty analizy (CoA)

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Dokumenty związane z niedawno zakupionymi produktami zostały zamieszczone w Bibliotece dokumentów.

Odwiedź Bibliotekę dokumentów

Connexin 43 a check-point component of cell proliferation implicated in a wide range of human testis diseases
Chevallier D, et al.
Cellular and Molecular Life Sciences, 70(7), 1207-1220 (2013)
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)
The gap junction channel protein connexin 43 is covalently modified and regulated by SUMOylation
Kjenseth A, et al.
The Journal of Biological Chemistry, 287(19), 15851-15861 (2012)
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)

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