Anti-GJB2抗体(ab65969)

概述

  • 产品名称Anti-GJB2抗体
    参阅全部 GJB2 一抗
  • 描述
    兔多克隆抗体to GJB2
  • 经测试应用适用于: WB, ICC, IHC-Pmore details
  • 种属反应性
    与反应: Mouse, Rat, Human
  • 免疫原

    A synthetic peptide corresponding to a sequence at the N-terminal of human Connexin 26, different from the related mouse and rat sequences by two amino acids.

  • 阳性对照
    • Rat liver lysate

性能

应用

Our Abpromise guarantee covers the use of ab65969 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
WB Use a concentration of 1 - 2 µg/ml. Detects a band of approximately 26 kDa (predicted molecular weight: 26 kDa).
ICC Use a concentration of 1 - 2 µg/ml.
IHC-P Use a concentration of 5 µg/ml. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.

靶标

  • 功能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.
  • 疾病相关Defects in GJB2 are the cause of deafness autosomal recessive type 1A (DFNB1A) [MIM:220290]. DFNB1A is a form of sensorineural hearing loss. Sensorineural deafness results from damage to the neural receptors of the inner ear, the nerve pathways to the brain, or the area of the brain that receives sound information.
    Defects in GJB2 are the cause of deafness autosomal dominant type 3A (DFNA3A) [MIM:601544].
    Defects in GJB2 are a cause of Vohwinkel syndrome (VS) [MIM:124500]. VS is an autosomal dominant disease characterized by hyperkeratosis, constriction on finger and toes and congenital deafness.
    Defects in GJB2 are a cause of palmoplantar keratoderma with deafness (PPKDFN) [MIM:148350]. PPKDFN is an autosomal dominant disorder characterized by the association of palmoplantar hyperkeratosis with progressive, bilateral, high-frequency, sensorineural deafness.
    Defects in GJB2 are a cause of keratitis-ichthyosis-deafness syndrome (KID syndrome) [MIM:148210]; an autosomal dominant form of ectodermal dysplasia. Ectodermal dysplasias (EDs) constitute a heterogeneous group of developmental disorders affecting tissues of ectodermal origin. EDs are characterized by abnormal development of two or more ectodermal structures such as hair, teeth, nails and sweat glands, with or without any additional clinical sign. Each combination of clinical features represents a different type of ectodermal dysplasia. KID syndrome is characterized by the association of hyperkeratotic skin lesions with vascularizing keratitis and profound sensorineural hearing loss. Clinical features include deafness, ichthyosis, photobia, absent or decreased eyebrows, sparse or absent scalp hair, decreased sweating and dysplastic finger and toenails.
    Defects in GJB2 are the cause of Bart-Pumphrey syndrome (BPS) [MIM:149200]. BPS is an autosomal dominant disorder characterized by sensorineural hearing loss, palmoplantar keratoderma, knuckle pads, and leukonychia, It shows considerable phenotypic variability.
    Defects in GJB2 are the cause of ichthyosis hystrix-like with deafness syndrome (HID syndrome) [MIM:602540]. HID syndrome is an autosomal-dominant inherited keratinizing disorder characterized by sensorineural deafness and spiky hyperkeratosis affecting the entire skin. HID syndrome is considered to differ from the similar KID syndrome in the extent and time of occurrence of skin symptoms and the severity of the associated keratitis.
  • 序列相似性Belongs to the connexin family. Beta-type (group I) subfamily.
  • 细胞定位Cell membrane. Cell junction > gap junction.
  • Information by UniProt
  • 数据库链接
  • 别名
    • connexin 26 antibody
    • Connexin-26 antibody
    • Cx26 antibody
    • CXB2_HUMAN antibody
    • DFNA3 antibody
    • DFNB1 antibody
    • Gap junction beta-2 protein antibody
    • GJB2 antibody
    • HID antibody
    • KID antibody
    • NSRD1 antibody
    • PPK antibody
    see all

Anti-GJB2 antibody 图像

  • Anti-GJB2 antibody (ab65969) at 1 µg/ml + Rat liver lysate

    Predicted band size : 26 kDa
    Observed band size : 26 kDa
  • IHC image of ab65969 staining in human normal colon formalin fixed paraffin embedded tissue section, performed on a Leica BondTM system using the standard protocol F. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH6, epitope retrieval solution 1) for 20 mins. The section was then incubated with ab65969, 5µg/ml, for 15 mins at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with haematoxylin and mounted with DPX.

    For other IHC staining systems (automated and non-automated) customers should optimize variable parameters such as antigen retrieval conditions, primary antibody concentration and antibody incubation times.

Anti-GJB2 antibody (ab65969)参考文献

ab65969 has not yet been referenced specifically in any publications.

Product Wall

Application Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Blocking step EnVision Flex Peroxidase-blocking reagent (ready-to-use - DAKO) as blocking agent for 15 minute(s) · Concentration: 1% · Temperature: 25°C
Antigen retrieval step Heat mediated - Buffer/Enzyme Used: Citrate buffer
Sample Cow Tissue sections (Fetal Liver)
Specification Fetal Liver
Permeabilization No
Fixative Formaldehyde
Username

Dr. Juliana Shimara Pires Ferrão

Verified customer

提交于 Nov 24 2014

Abcam has not validated the combination of species/application used in this Abreview.
Application Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Sample Horse Tissue sections (aglandular stomach, epithelium)
Antigen retrieval step Heat mediated - Buffer/Enzyme Used: citrate buffer
Permeabilization No
Specification aglandular stomach, epithelium
Blocking step Milk as blocking agent for 15 minute(s) · Concentration: 5% · Temperature: 22°C
Fixative Methacarn
Username

Dr. F Hernandez-Blazquez

Verified customer

提交于 Mar 16 2016

Abcam has not validated the combination of species/application used in this Abreview.
Application Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections)
Sample Monkey Tissue sections (Intestine)
Specification Intestine
Fixative Paraformaldehyde
Antigen retrieval step Heat mediated
Permeabilization No
Blocking step Protein Block, Serum-Free - Dako as blocking agent for 30 minute(s) · Concentration: 0.25%
Username

Abcam user community

Verified customer

提交于 Mar 29 2013

Thank you for contacting us and sorry for the delay in getting back to you.

The immunogen used to raise the Anti-GJB2 antibody (ab65969) is actually raised against a peptide taken from the N-terminal end of human GJB2. Not the middle region o...

Read More

Thank you for contacting us.

As your customer is running a sample of non-transfected HeLa cells as well, the absence of the 18 kDa band in this lane indicates that it is only an artefact due to the transfection. If it would be a cross-reactivi...

Read More

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"