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Product Name Connexin 26 Chinese Name 间隙连接蛋白26/GJB2抗体 Alias cx26; DFNA3; DFNB1; HID; KID; NSRD1; Connexin26; Connexin-26; GJB2; PPK; CXB2_HUMAN. Research Area Tumour Cell biology Neurobiology Signal transduction Immunogen Species Rabbit Clonality Polyclonal React Species Mouse, (predicted: Rat, ) Applications WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 26kDa Cellular localization The cell membrane Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from mouse Connexin 26 : 61-160/226 <Cytoplasmic> Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail Gap junction channels consist of connexin protein subunits, which are encoded by a multigene family. GJBs (gap junction proteins or connexins) play crucial functional roles associated with these channels. Immunohistochemical staining of human cochlear cells demonstrated high levels of GJB2 expression. Mutations in GJB2 are associated with genetically derived hearing impairments, including autosomal recessive nonsyndromic deafness.
Function:
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. May play a critical role in the physiology of hearing by participating in the recycling of potassium to the cochlear endolymph.
Subcellular Location:
Cell membrane. Cell junction, gap junction.
Tissue Specificity:
Expressed in the heart and fetal cochlea.
Post-translational modifications:
Belongs to the connexin family. Beta-type (group I) subfamily.
DISEASE:
Defects in GJA1 are the cause of autosomal dominant oculodentodigital dysplasia (ODDD) [MIM:164200]; also known as oculodentoosseous dysplasia. ODDD is a highly penetrant syndrome presenting with craniofacial (ocular, nasal, dental) and limb dysmorphisms, spastic paraplegia, and neurodegeneration. Craniofacial anomalies tipically include a thin nose with hypoplastic alae nasi, small anteverted nares, prominent columnella, and microcephaly. Brittle nails and hair abnormalities of hypotrichosis and slow growth are present. Ocular defects include microphthalmia, microcornea, cataracts, glaucoma, and optic atrophy. Syndactyly type 3 and conductive deafness can occur in some cases. Cardiac abnormalities are observed in rare instances. Defects in GJA1 are the cause of autosomal recessive oculodentodigital dysplasia (ODDD autosomal recessive) [MIM:257850].
Similarity:
Belongs to the connexin family. Alpha-type (group II) subfamily.
SWISS:
Q00977
Gene ID:
14619
Database links:
Entrez Gene: 2706 HumanEntrez Gene: 14619 Mouse
Omim: 121011 Human
SwissProt: P29033 Human
SwissProt: Q00977 Mouse
Unigene: 524894 Human
Unigene: 714494 Human
Unigene: 390683 Mouse
Unigene: 219269 Rat
间隙连接(Gap junction, GJ)是普遍存在于相邻细胞间的细胞连接方式,是相邻The cell membrane上的一种膜蛋白通道结构。间隙连接允许邻近细胞之间的离子,核苷酸和小的调节分子以及药物或它们的代谢物进行转运,细胞通过它所介导的细胞间连接通讯,进行细胞间信息和能量的传递,调控细胞的生长、分化和内环境的稳定,对维持机体的功能发挥重要作用. 间隙连接蛋白26目前主要用于Tumour方面的研究。Product Picture
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