骨骼肌慢肌肌钙蛋白T抗体
规格:1mg/1ml
英文名: TNNT1
别名: Troponin T slow skeletal muscle; TNNT1; TNNT1_HUMAN; Troponin T, slow skeletal muscle; TnTs; Slow skeletal muscle troponin T; sTnT; ANM; MGC104241; Slow skeletal muscle troponin T; sTnT.
分子量: 33kDa
储存液:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型:Polyclonal
亚型:IgG
纯化方法:affinity purified by Protein A
**原:KLH conjugated synthetic peptide derived from human TNNT1
交叉反应:Human,
细胞定位:
骨骼肌慢肌肌钙蛋白T抗体产品介绍:background: This gene encodes a protein that is a subunit of troponin, which is a regulatory complex located on the thin filament of the sarcomere. This complex regulates striated muscle contraction in response to fluctuations in intracellular calcium concentration. This complex is composed of three subunits: troponin C, which binds calcium, troponin T, which binds tropomyosin, and troponin I, which is an inhibitory subunit. This protein is the slow skeletal troponin T subunit. Mutations in this gene cause nemaline myopathy type 5, also known as Amish nemaline myopathy, a neuromuscular disorder characterized by muscle weakness and rod-shaped, or 骨骼肌慢肌肌钙蛋白T抗体nemaline, inclusions in skeletal muscle fibers which affects infants, resulting in death due to respiratory insufficiency, usually in the second year. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008] Function: Troponin T is the tropomyosin-binding subunit of troponin, the thin filament regulatory complex which confers calcium-sensitivity to striated muscle actomyosin ATPase activity. DISEASE: Nemaline myopathy 5 (NEM5) [MIM:605355]: A form of nemaline myopathy. Nemaline myopathies are muscular disorders characterized by muscle weakness of varying severity and onset, and abnormal thread-like or rod-shaped structures in muscle fibers on histologic examination. Nemaline myopathy type 5 is a severe and progressive form common among Old Order Amish. Affected infants display tremors 骨骼肌慢肌肌钙蛋白T抗体with hypotonia and mild contractures of the shoulders and hips. Proximal contractures progressively weaken and a pectus carinatum deformity develops before children die of respiratory insufficiency, usually in the second year. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the troponin T family. Gene ID: 7138 Database links: Entrez Gene: 7138 Human Omim: 191041 Human SwissProt: P13805 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
骨骼肌慢肌肌钙蛋白T抗体产品应用:WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (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.
研究领域:
储存条件: Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源: Rabbit
外观: Lyophilized or Liquid