GJB6 polyclonal antibody
产品名称: GJB6 polyclonal antibody
英文名称: GJB6 polyclonal antibody
产品编号: PAB17269
产品价格: null
产品产地: 台湾
品牌商标: Abnova
更新时间: null
使用范围:
亚诺法生技股份有限公司(Abnova)
- 联系人 :
- 地址 : 台湾台北市内湖区洲子街 108 号 9 楼
- 邮编 : 11493
- 所在区域 : 台湾
- 电话 : +886-920**1152 点击查看
- 传真 : 点击查看
- 邮箱 : sales@abnova.com.tw
- Specification
- Product Description:
- Goat polyclonal antibody raised against synthetic peptide of GJB6.
- Immunogen:
- A synthetic peptide corresponding to amino acids at internal region of human GJB6.
- Sequence:
- C-RSKRAQTQKNHPNH
- Host:
- Goat
- Theoretical MW (kDa):
- 30.4
- Specificity:
- Reported variants represent identical protein: NP_006774.2, NP_001103691.1, NP_001103689.1, NP_001103690.1
- Form:
- Liquid
- Purification:
- Antigen affinity purification
- Concentration:
- 0.5 mg/mL
- Storage Buffer:
- In Tris saline, pH 7.3 (0.5% BSA, 0.02% sodium azide)
- Storage Instruction:
- Store at -20°C.
Aliquot to avoid repeated freezing and thawing.
- Recommend Usage:
- ELISA (1:16000)
The optimal working dilution should be determined by the end user.
- Note:
- This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
- Publication Reference
- 1.
- Mutation in gap and tight junctions in patients with non-syndromic hearing loss.
Belguith H, Tlili A, Dhouib H, Ben Rebeh I, Lahmar I, Charfeddine I, Driss N, Ghorbel A, Ayadi H, Masmoudi S.Biochem Biophys Res Commun. 2009 Jul 17;385(1):1-5. Epub 2009 Feb 28.
- Application Image
- ELISA
- Entrez GeneID:
- 10804
- Protein Accession#:
- NP_006774.2
- Gene Name:
- GJB6
- Gene Alias:
- CX30,DFNA3,ED2,EDH,HED
- Gene Description:
- gap junction protein, beta 6, 30kDa
- Gene Ontology:
- Hyperlink
- Gene Summary:
- 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
- Other Designations:
- OTTHUMP00000018096,OTTHUMP00000176870,OTTHUMP00000176871,OTTHUMP00000176872,connexin 30,ectodermal dysplasia 2, hidrotic (Clouston syndrome),gap junction protein, beta 6,gap junction protein, beta 6 (connexin 30)
- Related Disease
- Auditory Perception
- Cholesteatoma
- Chronic Disease
- Cytomegalovirus Infections
- Deafness
- Deafness
- Disease Progression
- Genetic Predisposition to Disease
- Hearing Loss
- Hearing Loss, High-Frequency
- Hearing Loss, Noise-Induced
- Hearing Loss, Sensorineural
- Presbycusis
- Rhinitis
- Sinusitis
- Speech Perception
- Syndrome
- Usher Syndromes
- Vestibulocochlear Nerve Diseases