Product Name
Connexin-32 (GJB1), Polyclonal Antibody
Full Product Name
Connexin-32 Rabbit Polyclonal
Product Gene Name
anti-GJB1 antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Species Reactivity
Human, Mouse
Purity/Purification
>=95% as determined by SDS-PAGE
Immunogen Affinity Purified
Immunogen
Gap junction protein, beta 1, 32kDa
Preparation and Storage
PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Other Notes
Small volumes of anti-GJB1 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.
Applications Tested/Suitable for anti-GJB1 antibody
ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC), Immunoprecipitation (IP)
NCBI/Uniprot data below describe general gene information for GJB1. It may not necessarily be applicable to this product.
NCBI Accession #
AAA75086.1
[Other Products]
UniProt Secondary Accession #
Q5U0S4; B2R8R2; D3DVV2[Other Products]
UniProt Related Accession #
P08034[Other Products]
Molecular Weight
32,025 Da[Similar Products]
NCBI Official Full Name
connexin 32, partial
NCBI Official Synonym Full Names
gap junction protein beta 1
NCBI Official Symbol
GJB1??[Similar Products]
NCBI Official Synonym Symbols
CMTX; CX32; CMTX1
??[Similar Products]
NCBI Protein Information
gap junction beta-1 protein
UniProt Protein Name
Gap junction beta-1 protein
UniProt Synonym Protein Names
Connexin-32; Cx32; GAP junction 28 kDa liver protein
Protein Family
Gap junction beta-1 protein
UniProt Gene Name
GJB1??[Similar Products]
UniProt Synonym Gene Names
CX32; Cx32??[Similar Products]
UniProt Entry Name
CXB1_HUMAN
NCBI Summary for GJB1
This gene encodes a member of the gap junction protein family. The gap junction proteins are membrane-spanning proteins that assemble to form gap junction channels that facilitate the transfer of ions and small molecules between cells. According to sequence similarities at the nucleotide and amino acid levels, the gap junction proteins are divided into two categories, alpha and beta. Mutations in this gene cause X-linked Charcot-Marie-Tooth disease, an inherited peripheral neuropathy. Alternatively spliced transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Oct 2008]
UniProt Comments for GJB1
GJB1: 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. Defects in GJB1 are the cause of Charcot-Marie-Tooth disease X-linked type 1 (CMTX1); also designated CMT- X. CMTX1 is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot- Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies characterized by severely reduced motor nerve conduction velocities (NCVs) (less than 38m/s) and segmental demyelination and remyelination, and primary peripheral axonal neuropathies characterized by normal or mildly reduced NCVs and chronic axonal degeneration and regeneration on nerve biopsy. CMTX1 has both demyelinating and axonal features. Central nervous system involvement may occur. Defects in GJB1 may contribute to the phenotype of Dejerine-Sottas syndrome (DSS); also known as Dejerine-Sottas neuropathy (DSN) or hereditary motor and sensory neuropathy III (HMSN3). DSS is a severe degenerating neuropathy of the demyelinating Charcot-Marie-Tooth disease category, with onset by age 2 years. DSS is characterized by motor and sensory neuropathy with very slow nerve conduction velocities, increased cerebrospinal fluid protein concentrations, hypertrophic nerve changes, delayed age of walking as well as areflexia. There are both autosomal dominant and autosomal recessive forms of Dejerine- Sottas syndrome. Belongs to the connexin family. Beta-type (group I) subfamily.
Protein type: Motility/polarity/chemotaxis; Channel, misc.; Membrane protein, integral; Membrane protein, multi-pass
Chromosomal Location of Human Ortholog: Xq13.1
Cellular Component: connexon complex; endoplasmic reticulum membrane; integral to membrane
Molecular Function: gap junction channel activity; protein homodimerization activity
Biological Process: cell-cell signaling; gap junction assembly; nervous system development; protein oligomerization; purine ribonucleotide transport; transmembrane transport; transport
Disease: Charcot-marie-tooth Disease, X-linked Dominant, 1
Research Articles on GJB1
1. A novel point mutation in GJB1 was detected, expanding the spectrum of GJB1 mutations known to be associated with CMTX.
Precautions
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