Anti-Desmin抗体[RD301] (ab8976)
Key features and details
- Mouse monoclonal [RD301] to Desmin
- Suitable for: IHC-Fr
- Reacts with: Human, Zebrafish
- Isotype: IgG2b
概述
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产品名称
Anti-Desmin抗体[RD301]
参阅全部 Desmin 一抗 -
描述
小鼠单克隆抗体[RD301] to Desmin -
宿主
Mouse -
特异性
This antibody reacts exclusively with desmin, which is expressed in smooth and striated muscle cells and their tumors e.g. rhabdomyosarcoma and leiomyosarcoma. -
经测试应用
适用于: IHC-Frmore details -
种属反应性
与反应: Human, Zebrafish -
免疫原
Tissue, cells or virus corresponding to Chicken Desmin. Cytoskeletal desmin extract of chicken gizzard.
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常规说明
The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. Please check that this product meets your needs before purchasing.
If you have any questions, special requirements or concerns, please send us an inquiry and/or contact our Support team ahead of purchase. Recommended alternatives for this product can be found below, along with publications, customer reviews and Q&As
性能
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形式
Liquid -
存放说明
Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles. -
存储溶液
Preservative: 0.09% Sodium azide
Constituent: PBS -
Concentration information loading...
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纯度
Protein A purified -
克隆
单克隆 -
克隆编号
RD301 -
骨髓瘤
Sp2/0-Ag14 -
同种型
IgG2b -
轻链类型
unknown -
研究领域
相关产品
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Compatible Secondaries
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Isotype control
应用
应用 | Ab评论 | 说明 |
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IHC-Fr |
1/100 - 1/2000. with avidin-biotinylated horseradish peroxidase complex (ABC) as detection reagent.
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说明 |
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IHC-Fr
1/100 - 1/2000. with avidin-biotinylated horseradish peroxidase complex (ABC) as detection reagent. |
靶标
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功能
Desmin are class-III intermediate filaments found in muscle cells. In adult striated muscle they form a fibrous network connecting myofibrils to each other and to the plasma membrane from the periphery of the Z-line structures. -
疾病相关
Defects in DES are the cause of myopathy myofibrillar desmin-related (MFM-DES) [MIM:601419]; also known as desmin-related myopathy (DRM). A neuromuscular disorder characterized by skeletal muscle weakness associated with cardiac conduction blocks, arrhythmias, restrictive heart failure, and by myofibrillar destruction with intracytoplasmic accumulation of desmin-reactive deposits in cardiac and skeletal muscle cells.
Defects in DES are the cause of cardiomyopathy dilated type 1I (CMD1I) [MIM:604765]. Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.
Defects in DES are the cause of neurogenic scapuloperoneal syndrome Kaeser type (Kaeser syndrome) [MIM:181400]. Kaeser syndrome is an autosomal dominant disorder with a peculiar scapuloperoneal distribution of weakness and atrophy. A large clinical variability is observed ranging from scapuloperoneal, limb grindle and distal phenotypes with variable cardiac or respiratory involvement. Facial weakness, dysphagia and gynaecomastia are frequent additional symptoms. Affected men seemingly bear a higher risk of sudden, cardiac death as compared to affected women. Histological and immunohistochemical examination of muscle biopsy specimens reveal a wide spectrum of findings ranging from near normal or unspecific pathology to typical, myofibrillar changes with accumulation of desmin. -
序列相似性
Belongs to the intermediate filament family. -
细胞定位
Cytoplasm. - Information by UniProt
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数据库链接
- Entrez Gene: 1674 Human
- Omim: 125660 Human
- SwissProt: P17661 Human
- Unigene: 594952 Human
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别名
- CMD1I antibody
- CSM1 antibody
- CSM2 antibody
see all
图片
数据表及文件
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Datasheet download
文献 (14)
ab8976 被引用在 14 文献中.
- Matsumura G et al. Evaluation method for cell-free in situ tissue-engineered vasculature monitoring: Proof of growth and development in a canine IVC model. PLoS One 17:e0267274 (2022). PubMed: 35436313
- Ji G et al. Case Report: A Novel Splice-Site Mutation in DNAJB6 Associated With Juvenile-Onset Proximal-Distal Myopathy in a Chinese Patient. Front Genet 13:925926 (2022). PubMed: 35812750
- Zhang XZ et al. Procyanidins-crosslinked small intestine submucosa: A bladder patch promotes smooth muscle regeneration and bladder function restoration in a rabbit model. Bioact Mater 6:1827-1838 (2021). PubMed: 33336114
- Goto O et al. Novel Method of Sampling the Gastrointestinal Muscle Layer: Feasibility of Endoscopic Muscular Resection with a Ligation Device in an in Vivo Porcine Model. J Nippon Med Sch 88:441-447 (2021). PubMed: 34789604
- Luca E et al. Genetic deletion of microRNA biogenesis in muscle cells reveals a hierarchical non-clustered network that controls focal adhesion signaling during muscle regeneration. Mol Metab 36:100967 (2020). PubMed: 32240622