HPA021004
Anti-MYBPC1 antibody produced in rabbit
Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
别名:
Anti-C-protein, skeletal muscle slow isoform, Anti-Myosin-binding protein C, slow-type, Anti-Slow MyBP-C
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所有图片(6)
About This Item
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生物源
rabbit
品質等級
共軛
unconjugated
抗體表格
affinity isolated antibody
抗體產品種類
primary antibodies
無性繁殖
polyclonal
產品線
Prestige Antibodies® Powered by Atlas Antibodies
形狀
buffered aqueous glycerol solution
物種活性
human
加強驗證
independent
orthogonal RNAseq
Learn more about Antibody Enhanced Validation
技術
immunohistochemistry: 1:50- 1:200
免疫原序列
DWTLVETPPGEEQAKQNANSQLSILFIEKPQGGTVKVGEDITFIAKVKAEDLLRKPTIKWFKGKWMDLASKAGKHLQLKETFERHSRVYTFEMQIIKAKDNFAGNYRCEVTYKDKFDSCSFDLEVHESTGTTPN
UniProt登錄號
運輸包裝
wet ice
儲存溫度
−20°C
目標翻譯後修改
unmodified
基因資訊
human ... MYBPC1(4604)
一般說明
The gene MYBPC1 (myosin-binding protein C, slow type) is mapped to human chromosome 12q23.2.
免疫原
Myosin-binding protein C, slow-type recombinant protein epitope signature tag (PrEST)
應用
Anti-MYBPC1 antibody produced in rabbit, a Prestige Antibody, is developed and validated by the Human Protein Atlas (HPA) project . Each antibody is tested by immunohistochemistry against hundreds of normal and disease tissues. These images can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. The antibodies are also tested using immunofluorescence and western blotting. To view these protocols and other useful information about Prestige Antibodies and the HPA, visit sigma.com/prestige. In addition, the antibody has been used for immunocytochemistry.
生化／生理作用
MYBPC1 (myosin-binding protein C, slow type) is a proteasomal substrate. It associates with myosin and titin, and is suggested to stabilize sarcomere structure. In addition, MYBPC1 interaction with myosin-S2 might be crucial for regulation of muscle contraction. In muscles, MTBPC1 association with myosin and MM-CK (muscle-type creatine kinase) controls energy homoeostasis. It is associated with distal arthrogryposis type I (DA1) and lethal congenital contractural syndrome type 4 (LCCS4). Mutation in MYBPC1 is cause of hypertrophic cardiomyopathy, linked with left ventricular dysfunction and dilation.
特點和優勢
Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.
Every Prestige Antibody is tested in the following ways:
Every Prestige Antibody is tested in the following ways:
- IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
- Protein array of 364 human recombinant protein fragments.
聯結
Corresponding Antigen APREST72729
外觀
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
法律資訊
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
免責聲明
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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儲存類別代碼
10 - Combustible liquids
水污染物質分類（WGK）
WGK 1
閃點（°F）
Not applicable
閃點（°C）
Not applicable
Gabriele Tonni et al.
Congenital anomalies, 53(3), 137-140 (2013-01-30)
An early second-trimester prenatal ultrasound diagnosis of an arthrogryposis multiplex congenita-like syndrome associated with median clefts is reported. A molecular biological work-up was performed to search for a potentially overlapping syndrome and dysostosis. Autopsy and postmortem radiogram were performed to
Saba Abdul-Hussein et al.
BMC musculoskeletal disorders, 13, 262-262 (2013-01-01)
The formation of contractile myofibrils requires the stepwise onset of expression of muscle specific proteins. It is likely that elucidation of the expression patterns of muscle-specific sarcomeric proteins is important to understand muscle disorders originating from defects in contractile sarcomeric
Barak Markus et al.
Human mutation, 33(10), 1435-1438 (2012-05-23)
Autosomal recessive lethal congenital contractural syndrome (LCCS) is a severe form of neuromuscular arthrogryposis. We previously showed that this phenotype is caused in two unrelated inbred Bedouin tribes by different defects in the phosphatidylinositol pathway. However, the molecular basis of
Tetsuo Konno et al.
Journal of the American College of Cardiology, 41(5), 781-786 (2003-03-12)
We studied the clinical features of hypertrophic cardiomyopathy (HCM) caused by a novel mutation in the myosin binding protein-C (MyBP-C) gene in patients and family members of Japanese descent. Previous reports have demonstrated that the clinical features of HCM associated
Christina A Gurnett et al.
Human molecular genetics, 19(7), 1165-1173 (2010-01-05)
Distal arthrogryposis type I (DA1) is a disorder characterized by congenital contractures of the hands and feet for which few genes have been identified. Here we describe a five-generation family with DA1 segregating as an autosomal dominant disorder with complete
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