MYBPC1 Antibody, HRP conjugated

說明書

中文名稱：

MYBPC1兔多克隆抗體, HRP偶聯
貨號：

CSB-PA717389LB01HU
規格：

￥880
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) MYBPC1 Polyclonal antibody
Uniprot No.：

Q00872
基因名：

MYBPC1
別名：

C protein, skeletal muscle slow isoform antibody; C-protein antibody; MYBPC1 antibody; MYBPCC antibody; MYBPCS antibody; Myosin binding protein C, slow type antibody; Myosin-binding protein C antibody; MYPC1_HUMAN antibody; skeletal muscle C protein antibody; skeletal muscle slow isoform antibody; Slow MyBP C antibody; Slow MyBP-C antibody; slow-type antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Myosin-binding protein C, slow-type protein (618-779AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

HRP
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
產品提供形式：

Liquid
應用范圍：

ELISA
Protocols：

ELISA Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

功能：

Thick filament-associated protein located in the crossbridge region of vertebrate striated muscle a bands. Slow skeletal protein that binds to both myosin and actin. In vitro, binds to native thin filaments and modifies the activity of actin-activated myosin ATPase. May modulate muscle contraction or may play a more structural role.
基因功能參考文獻：
1. A novel milder MYBPC1 homozygous phenotype causes arthrogryposis multiplex congenita in a consanguineous Israeli Druze pedigree. PMID: 26661508
2. Ca(2+) modulates the interaction of cMyBP-C with the thin filament in the sarcomere. PMID: 26831109
3. Two novel mutations in myosin binding protein C slow causing distal arthrogryposis type 2 were both found to occur in the C2 immunoglobulin domain, which constitutes part of the binding site for the S2 subfragment of myosin. PMID: 25679999
4. Mutations in the MYH7 gene, rather than in the MYBPC3 gene, were also related to a worse prognosis. This is the first work characterizing HC molecular epidemiology in the Brazilian population for the 3 most important genes. PMID: 24093860
5. Autosomal recessive lethal congenital contractural syndrome type 4 (LCCS4) caused by a mutation in MYBPC1 PMID: 22610851
6. Significant molecule MYBPC1 phosphoprotein network from 12 frontal cortex of HIV encephalitis (HIVE) control patients and 16 HIVE, was identified and constructed. PMID: 21061152
7. MyBPC1 acts as an adaptor to connect the ATP consumer (myosin) and the regenerator (muscle type creatine kinase) for efficient energy metabolism and homoeostasis. PMID: 21426302
8. These findings reveal that the MYBPC1 is a novel gene responsible for DA1, though the mechanism of disease may differ from how some cardiac MYBPC3 mutations cause hypertrophic cardiomyopathy. PMID: 20045868
9. Screening patients with dilated cardiomyopathy, as well as hypertrophic cardiomyopathy, for this mutation is of signifiant importance with this mutation diagnosing dilated cardiomyopathy. PMID: 12628722
10. The present study demonstrates slow skeletal muscle type C-protein in moderate amount in right atrium and interatrial septum of adult human, rabbit, rat and bovine hearts using both immunocytochemical and immunoblotting procedures. PMID: 16003462
11. to determine whether HCM mutations in beta myosin heavy chain located within the light meromyosin portion alter the binding of cMyBP-C, and to define the precise region of this binding. PMID: 16918501
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相關疾病：

Arthrogryposis, distal, 1B (DA1B); Lethal congenital contracture syndrome 4 (LCCS4)
蛋白家族：

Immunoglobulin superfamily, MyBP family
數據庫鏈接：

HGNC: 7549

OMIM: 160794

KEGG: hsa:4604

STRING: 9606.ENSP00000354849

UniGene: Hs.654589

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