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anti-GJB1 antibody :: Rabbit Connexin 32/GJB1 Polyclonal Antibody

Scan QR to view Datasheet Catalog #    MBS175229
Unit / Price
0.1 mg  /  $245 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

Connexin 32/GJB1, Polyclonal Antibody

 Also Known As   

Anti-Connexin 32/GJB1 antibody

 Product Synonym Names    Gap junction beta-1 protein; gap junction protein, beta 1, 32kDa; Charcot Marie Tooth neuropathy X linked antibody; CMTX 1 antibody; CMTX antibody; CMTX1 antibody; Connexin-32 antibody; Connexin32 antibody; CX 32 antibody; CX32 antibody; CXB1_HUMAN antibody; GAP junction 28 kDa liver protein antibody; Gap junction beta 1 protein antibody; Gap junction beta-1 protein antibody; Gap junction protein beta 1 32kD antibody; Gap junction protein beta 1 antibody; GJB 1 antibody; GJB1 antibody
 Product Gene Name   

anti-GJB1 antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 OMIM    304040
 3D Structure    ModBase 3D Structure for P08034
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 Clonality    Polyclonal
 Isotype    IgG
 Host    Rabbit
 Species Reactivity    Human, Mouse, Rat
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 Purity/Purification    Immunogen affinity purified.
 Form/Format    Lyophilized
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 Immunogen    A synthetic peptide corresponding to a sequence in the middle region of human Connexin 32/GJB1 (215-231aa RACARRAQRRSNPPSRK), identical to the related mouse and rat sequences.
 Contents    Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3.
 Reconstitution    Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
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 Preparation and Storage    At -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for a longer time. Avoid repeated freezing and thawing.
 ISO Certification    Manufactured in an ISO 9001:2008 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.
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Product Description specifically for anti-GJB1 antibody

   Description: Rabbit IgG polyclonal antibody for Gap junction beta-1 protein(GJB1) detection. Tested with WB, IHC-P in Human, Mouse, Rat.
Background: Connexins are membrane-spanning proteins that assemble to form gap junction channels that facilitate the transfer of ions and small molecules between cells(Bergoffen et al., 1993). For a general discussion of connexin proteins, also known as Gap junction beta-1 protein. Gap junction beta-1 protein is a protein that in humans is encoded by the GJB1 gene. In melanocytic cells GJB1 gene expression may be regulated by MITF.
 Applications Tested/Suitable for anti-GJB1 antibody   

Western Blot (WB), Immunohistochemistry (IHC) Paraffin

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 Testing Data of anti-GJB1 antibody    anti-GJB1 antibody Testing Data image
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 Testing Data #2 of anti-GJB1 antibody    anti-GJB1 antibody Testing Data #2 image
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NCBI/Uniprot data below describe general gene information for GJB1. It may not necessarily be applicable to this product.
 NCBI GI #    117688
 NCBI GeneID    2705
 NCBI Accession #    P08034.1 [Other Products]
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 UniProt Primary Accession #    P08034 [Other Products]
 UniProt Secondary Accession #    Q5U0S4; B2R8R2; D3DVV2 [Other Products]
 UniProt Related Accession #    P08034 [Other Products]
 Molecular Weight    32,025 Da
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 NCBI Official Full Name    Gap junction beta-1 protein
 NCBI Official Synonym Full Names    gap junction protein, beta 1, 32kDa
 NCBI Official Symbol    GJB1 [Similar Products]
 NCBI Official Synonym Symbols   
CMTX; CX32; CMTX1
[Similar Products]
 NCBI Protein Information    gap junction beta-1 protein; connexin 32; connexin-32; GAP junction 28 kDa liver 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
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 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]
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 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; Membrane protein, multi-pass; Membrane protein, integral; Channel, misc.

Chromosomal Location of Human Ortholog: Xq13.1

Cellular Component: connexon complex; endoplasmic reticulum membrane; integral to membrane

Molecular Function: protein homodimerization activity

Biological Process: nervous system development; gap junction assembly; cell-cell signaling; transport

Disease: Charcot-marie-tooth Disease, X-linked Dominant, 1
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 Research Articles on GJB1    1. Mutations in connexin 32 gene in patients with chronic rhinosinusitis, including recurrent acute rhinosinusitis, appear to be rare
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with anti-GJB1 antibodyPathways associated with anti-GJB1 antibody
 Reference Product  PubMed Publications
 MPZ antibody  >49 publications with GJB1 and MPZ
 PMP22 antibody  >47 publications with GJB1 and PMP22
 EGR2 antibody  >23 publications with GJB1 and EGR2
 GJA1 antibody  >14 publications with GJB1 and GJA1
 GJB2 antibody  >13 publications with GJB1 and GJB2
 SOX10 antibody  >8 publications with GJB1 and SOX10
 GJD3 antibody  >1 publications with GJB1 and GJD3
 Products by Pathway  Pathway Diagram
 Calcium Regulation In The Cardiac Cell Pathway antibodies  Calcium Regulation In The Cardiac Cell Pathway Diagram
 Gap Junction Assembly Pathway antibodies  Gap Junction Assembly Pathway Diagram
 Gap Junction Trafficking Pathway antibodies  Gap Junction Trafficking Pathway Diagram
 Gap Junction Trafficking And Regulation Pathway antibodies  Gap Junction Trafficking And Regulation Pathway Diagram
 Membrane Trafficking Pathway antibodies  Membrane Trafficking Pathway Diagram
 Neural Crest Differentiation Pathway antibodies  Neural Crest Differentiation Pathway Diagram
 Oligomerization Of Connexins Into Connexons Pathway antibodies  Oligomerization Of Connexins Into Connexons Pathway Diagram
 Transport Of Connexins Along The Secretory Pathway antibodies  Transport Of Connexins Along The Secretory Pathway Diagram
Diseases associated with anti-GJB1 antibodyOrgans/Tissues associated with anti-GJB1 antibody
 Disease Name  Pubmed Publications
 Brain Diseases Antibodies  >12 publications with GJB1 and Brain Diseases
 Charcot-Marie-Tooth disease, X-linked, 1 Antibodies  >8 publications with GJB1 and Charcot-Marie-Tooth disease, X-linked, 1
 Disease Models, Animal Antibodies  >6 publications with GJB1 and Disease Models, Animal
 Edema Antibodies  >2 publications with GJB1 and Edema
 Inflammation Antibodies  >2 publications with GJB1 and Inflammation
 Infertility, Male Antibodies  >1 publications with GJB1 and Infertility, Male
 Neoplasms, Experimental Antibodies  >1 publications with GJB1 and Neoplasms, Experimental
 Urinary Bladder Neoplasms Antibodies  >1 publications with GJB1 and Urinary Bladder Neoplasms
 Liver Diseases Antibodies  >1 publications with GJB1 and Liver Diseases
 Fatty Liver Antibodies  >1 publications with GJB1 and Fatty Liver
 Organ/Tissue Name  Pubmed Publications
 Brain Antibodies  >19 publications with GJB1 and Brain
 Liver Antibodies  >5 publications with GJB1 and Liver
 Heart Antibodies  >2 publications with GJB1 and Heart
 Skin Antibodies  >2 publications with GJB1 and Skin
 Embryonic Tissue Antibodies  >1 publications with GJB1 and Embryonic Tissue
 Mammary Gland Antibodies  >1 publications with GJB1 and Mammary Gland
 Pancreas Antibodies  >1 publications with GJB1 and Pancreas
 Kidney Antibodies  >1 publications with GJB1 and Kidney
 Testis Antibodies  >1 publications with GJB1 and Testis
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