DMD monoclonal antibody, clone SPM499
产品名称： DMD monoclonal antibody, clone SPM499
英文名称： DMD monoclonal antibody, clone SPM499
产品编号： MAB2112
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:
- Mouse monoclonal antibody raised against synthetic peptide of DMD.
- Immunogen:
- A synthetic peptide corresponding to C-terminus of human DMD.
- Host:
- Mouse
- Theoretical MW (kDa):
- 427
- Reactivity:
- Human
- Form:
- Liquid
- Purification:
- Protein G purification
- Concentration:
- Lot specific
- Isotype:
- IgG1, kappa
- Storage Buffer:
- In 10 mM PBS, pH 7.4 (1% BSA, 0.1% sodium azide)
- Storage Instruction:
- Store at 4°C.
- Quality Control Testing:
- Antibody Reactive Against Synthetic Peptide.
- Recommend Usage:
- Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) (Formalin/PFA-fixed paraffin-embedded sections) (1:50)
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.
- Entrez GeneID:
- 1756
- Gene Name:
- DMD
- Gene Alias:
- BMD,CMD3B,DXS142,DXS164,DXS206,DXS230,DXS239,DXS268,DXS269,DXS270,DXS272
- Gene Description:
- dystrophin
- Gene Ontology:
- Hyperlink
- Gene Summary:
- The dystrophin gene is the largest gene found in nature, measuring 2.4 Mb. The gene was identified through a positional cloning approach, targeted at the isolation of the gene responsible for Duchenne (DMD) and Becker (BMD) Muscular Dystrophies. DMD is a recessive, fatal, X-linked disorder occurring at a frequency of about 1 in 3,500 new-born males. BMD is a milder allelic form. In general, DMD patients carry mutations which cause premature translation termination (nonsense or frame shift mutations), while in BMD patients dystrophin is reduced either in molecular weight (derived from in-frame deletions) or in expression level. The dystrophin gene is highly complex, containing at least eight independent, tissue-specific promoters and two polyA-addition sites. Furthermore, dystrophin RNA is differentially spliced, producing a range of different transcripts, encoding a large set of protein isoforms. Dystrophin (as encoded by the Dp427 transcripts) is a large, rod-like cytoskeletal protein which is found at the inner surface of muscle fibers. Dystrophin is part of the dystrophin-glycoprotein complex (DGC), which bridges the inner cytoskeleton (F-actin) and the extra-cellular matrix. [provided by RefSeq
- Other Designations:
- Duchenne muscular dystrophy protein,OTTHUMP00000023117,OTTHUMP00000023124,OTTHUMP00000023125,OTTHUMP00000023126,muscular dystrophy, Duchenne and Becker types
- Related Disease
- Bone Marrow Diseases
- Cardiomyopathy, Dilated
- Cardiovascular Diseases
- Cognition
- Cognition Disorders
- Diabetes Mellitus, Type 2
- Duchenne muscular dystrophy
- Edema
- Genetic Predisposition to Disease
- Intelligence Tests
- Mental Retardation
- Muscular Dystrophies
- Muscular Dystrophy, Duchenne
- Neuropsychological Tests
- Ventricular Dysfunction, Left