GJB6, Polyclonal Antibody

ED2, EDH, HED, CX30, HED2, DFNA3, ECTD2, DFNA3B, DFNB1B

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

Antigen affinity purification

1.1mg/mL (lot specific)

Store at -20 degree C (regular) and -80 degree C (long term). Avoid freeze / thaw cycles.

Manufactured in an ISO 9001:2015 Certified Laboratory.

Small volumes of anti-GJB6 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Gap junctions allow the transport of ions and metabolites between the cytoplasm of adjacent cells. They are formed by two hemichannels, made up of six connexin proteins assembled in groups. Each connexin protein has four transmembrane segments, two extracellular loops, a cytoplasmic loop formed between the two inner transmembrane segments, and the N- and C-terminus both being in the cytoplasm. The specificity of the gap junction is determined by which connexin proteins comprise the hemichannel. In the past, connexin protein names were based on their molecular weight, however the new nomenclature uses sequential numbers based on which form (alpha or beta) of the gap junction is present. This gene encodes one of the connexin proteins. Mutations in this gene have been found in some forms of deafness and in some families with hidrotic ectodermal dysplasia.

ELISA (EIA), Immunohistochemistry (IHC)

IHC: 1:25-1:100

Immunohistochemistry of paraffin-embedded Human tonsil tissue using GJB6 Polyclonal Antibody at dilution 1:40

30,387 Da

gap junction protein, beta 6, 30kDa

gap junction beta-6 protein; connexin 30; connexin-30; ectodermal dysplasia 2, hidrotic (Clouston syndrome); gap junction protein, beta 6 (connexin 30)

Gap junction beta-6 protein

Cx30  [Similar Products]

CXB6_HUMAN

Gap junctions allow the transport of ions and metabolites between the cytoplasm of adjacent cells. They are formed by two hemichannels, made up of six connexin proteins assembled in groups. Each connexin protein has four transmembrane segments, two extracellular loops, a cytoplasmic loop formed between the two inner transmembrane segments, and the N- and C-terminus both being in the cytoplasm. The specificity of the gap junction is determined by which connexin proteins comprise the hemichannel. In the past, connexin protein names were based on their molecular weight, however the new nomenclature uses sequential numbers based on which form (alpha or beta) of the gap junction is present. This gene encodes one of the connexin proteins. Mutations in this gene have been found in some forms of deafness and in some families with hidrotic ectodermal dysplasia. [provided by RefSeq, Jul 2008]

GJB6: 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. Belongs to the connexin family. Beta-type (group I) subfamily. A connexon is composed of a hexamer of connexins. Interacts with CNST

Protein type: Membrane protein, multi-pass

Chromosomal Location of Human Ortholog: 13q12

Cellular Component: connexon complex; apical plasma membrane; integral to membrane; cytosol

Biological Process: negative regulation of cell proliferation; sensory perception of sound; apoptosis; cell communication; response to lipopolysaccharide; ear morphogenesis; response to electrical stimulus; inner ear development; aging

Disease: Deafness, Autosomal Recessive 1a; Clouston Syndrome; Deafness, Autosomal Recessive 1b; Deafness, X-linked 2; Deafness, Autosomal Dominant 3b

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