Anti-Myotilin抗体上海钰博生物科技有限公司

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产品名称: Anti-Myotilin抗体
英文名称: Myotilin
抗体货号: YB--6479R
产品规格: 20ul/50ul/100ul/200ul
级    别: 分析纯, 见说明书
产品产地: 中国/美国
品牌商标: Ybscience
价    格: 1580元
抗原: KLH conjugated synthetic peptide derived from human Myotilin:121-200/498
抗原来源: Rabbit
抗体来源: Rabbit
适用物种: Human, Mouse, Rat, Dog, Pig, Cow, Horse,
见说明书conjugate:见说明书
Isotype:见说明书
应用范围: 科研使用
更新时间: 2019/6/6 11:01:00
详细资料:  实验方法技术资料
浏览人数:31
诚信指数:1658点
了解更多:进入公司展台
使用范围:仅限科研使用,不能应用于临床
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上海钰博生物科技有限公司
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产品详细描述

 Anti-Myotilin抗体

产品编号 YB-6479R
英文名称 Myotilin
中文名称 肌收缩蛋白MYOT抗体
别    名 57 kDa cytoskeletal protein; LGMD 1; LGMD1; Myofibrillar titin like Ig domains protein; Myofibrillar titin-like Ig domains protein; MYOT; MYOTI_HUMAN; Myotilin; Titin immunoglobulin domain protein; TTID; TTID protein.  
规格价格 100ul/1580元 购买    200ul/2480元 购买    大包装/询价
说 明 书 100ul  200ul
研究领域 心血管  细胞生物  神经生物学  信号转导  细胞骨架  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Horse, 
产品应用 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 IF=1:50-200 (石蜡切片需做抗原修复) 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 55kDa
细胞定位 细胞浆 细胞膜 
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Myotilin:121-200/498 
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
PubMed PubMed
产品介绍 background:
Myotilin, a sarcomeric protein that is encoded by the gene mapping to human chromosome 5q31, binds to a-actinin and is localized in the Z-line of myofibrils. Myotilin is expressed in skeletal and cardiac muscle, and it co-localizes with a-actinin in the sarcomeric I-bands where it directly interacts with a-actinin. Defects in the myotilin gene are reported to cause a form of autosomal dominant limb-girdle muscular dystrophy (LGMD). Symptoms of adult onset LGMD are progressive weakness of the hip and shoulder girdles as well as a distinctive dysarthric pattern of speech. The muscle of affected individuals with LGMD shows degeneration of myofibers, variations in fiber size, fiber splitting, centrally located myonuclei and an enhanced number of autophagic vesicles. 

Function:
Component of a complex of multiple actin cross-linking proteins. Involved in the control of myofibril assembly and stability at the Z lines in muscle cells.

Subunit:
Homodimer. Interacts with ACTA1, ACTN1, FLNA, FLNB, FLNC and MYOZ2. Interacts with the C-terminal region of MYOZ1.

Subcellular Location:
Cell membrane, sarcolemma. Cytoplasm, cytoskeleton. Cytoplasm, myofibril, sarcomere, Z line.

Tissue Specificity:
Expressed in skeletal muscle (at protein level). Expressed in skeletal muscle, heart, bone marrow and thyroid gland.

DISEASE:
Defects in MYOT are the cause of limb-girdle muscular dystrophy type 1A (LGMD1A). LGMD1A is an autosomal dominant degenerative myopathy with onset within a mean age of 28 years. LGMD1A is characterized by progressive skeletal muscle weakness of the hip and shoulder girdles, later progressing to include distal weakness, as well as a distinctive dysarthric pattern of speech. Affected muscle exhibits disorganization and streaming of the Z-line.
Defects in MYOT are the cause of myopathy myofibrillar type 3 (MFM3) [MIM:609200]. A neuromuscular disorder characterized by progressive skeletal muscle weakness greater distally than proximally, tight heel cords, hyporeflexia, cardiomyopathy and peripheral neuropathy in some patients. Affected muscle exhibits disorganization and streaming of the Z-line, presence of large hyaline structures, excessive accumulation of myotilin and other ectopically expressed proteins and prominent congophilic deposits.
Defects in MYOT are the cause of spheroid body myopathy (SBM) [MIM:182920]. SBM is an autosomal dominant form of myofibrillar myopathy (MFM), characterized by slowly progressing proximal muscle weakness and dysarthric nasal speech. There is no evidence of cardiomyopathy. Muscle biopsy shows spheroid bodies within the type I muscle fibers.

Similarity:
Belongs to the myotilin/palladin family.
Contains 2 Ig-like C2-type (immunoglobulin-like) domains.

SWISS:
Q9UBF9

Gene ID:
9499

Database links:

Entrez Gene: 9499 Human

Entrez Gene: 58916 Mouse

Entrez Gene: 291605 Rat

Omim: 604103 Human

SwissProt: Q9UBF9 Human

SwissProt: Q9JIF9 Mouse

Unigene: 84665 Human

Unigene: 143804 Mouse

Unigene: 163370 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications

生物在线声明:以上所展示的信息由企业自行提供,内容的真实性、准确性和合法性由发布企业负责。生物在线对此不承担任何保证责任。

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