γ-原肌球蛋白/原肌球蛋白3抗体
规格：1mg/1ml
英文名： Tropomyosin 3
别名： Alpha tropomyosin 3; Alpha tropomyosin slow skeletal; CFTD; Cytoskeletal tropomyosin TM30; FLJ41118; gamma TM; Gamma tropomyosin; Gamma-tropomyosin; Heat stable cytoskeletal protein 30 kDa; hscp30; hT
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 Tropomyo
交叉反应：Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse,
细胞定位：细胞浆
γ-原肌球蛋白/原肌球蛋白3抗体产品介绍：background: This gene encodes a member of the tropomyosin family of actin-binding proteins. Tropomyosins are dimers of coiled-coil proteins that provide stability to actin filaments and regulate access of other actin-binding proteins. Mutations in this gene result in autosomal dominant nemaline myopathy and other muscle disorders. This locus is involved in translocations with other loci, including anaplastic lymphoma receptor tyrosine kinase (ALK) and neurotrophic tyrosine kinase receptor type 1 (NTRK1), which result in the formation of fusion proteins that act as oncogenes. There are numerous pseudogenes for this gene on different chromosomes. Alternative splicing results in multiple transcript variants. [provided by RefSeq, May 2013] Function: Binds to actin filaments in muscle and non-muscle cells. Plays a central role, in association with the troponin complex, in the calcium dependent regulation of vertebrate striated muscle contraction. Smooth muscle contraction is regulated by interaction with caldesmon. In non-muscle cells is implicated in stabilizing cytoskeleton actin filaments. Subcellular Location: Cytoplasm > cytoskeleton. DISEASE: Defects in TPM3 are the cause of nemaline myopathy type 1 (NEM1) [MIM:609284].γ-原肌球蛋白/原肌球蛋白3抗体 A form of nemaline myopathy with autosomal dominant or recessive inheritance. Nemaline myopathies are muscular disorders characterized by muscle weakness of varying severity and onset, and abnormal thread-or rod-like structures in muscle fibers on histologic examination. Autosomal dominant nemaline myopathy type 1 is characterized by a moderate phenotype with onset between birth and early second decade of life. Weakness is diffuse and symmetric with slow progression often with need for a wheelchair in *****hood. The autosomal recessive form has onset at birth with moderate-to-severe hypotonia and diffuse weakness. In the most severe cases, death can occur before 2 years. Less severe cases have delayed major motor milestones, and these patients may walk, but often need a wheelchair before 10 years. Defects in TPM3 are a cause γ-原肌球蛋白/原肌球蛋白3抗体of thyroid papillary carcinoma (TPC) [MIM:188550]. TPC is a common tumor of the thyroid that typically arises as an irregular, solid or cystic mass from otherwise normal thyroid tissue. Papillary carcinomas are malignant neoplasm characterized by the formation of numerous, irregular, finger-like projections of fibrous stroma that is covered with a surface layer of neoplastic epithelial cells. Note=A chromosomal aberration involving TPM3 is found in thyroid papillary carcinomas. A rearrangement with NTRK1 generates the TRK fusion transcript by fusing the amino end of isoform 2 of TPM3 to the 3'-end of NTRK1. Similarity: Belongs to the tropomyosin family. Gene ID: 7170 Database links: Entrez Gene: 7170 Human Entrez Gene: 59069 Mouse Entrez Gene: 414388 Pig Entrez Gene: 117557 Rat Omim: 191030 Human SwissProt: P06753 Human SwissProt: P21107 Mouse SwissProt: A1XQV4 Pig SwissProt: Q63610 Rat Unigene: 535581 Human Unigene: 644306 Human Unigene: 654421 Human Unigene: 240839 Mouse Unigene: 421791 Mouse Unigene: 37575 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
γ-原肌球蛋白/原肌球蛋白3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：肿瘤 细胞生物 信号转导 结合蛋白 细胞骨架
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid