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Rabbit Anti-Tropomyosin 3/PE Conjugated antibody (bs-17158R-PE)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-17158R-PE
英文名稱 Rabbit Anti-Tropomyosin 3/PE Conjugated antibody
中文名稱 PE標記的γ-原肌球蛋白/原肌球蛋白3抗體
別    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; hTM30nm; hTM5; hTMnm; MGC102590; MGC14582; MGC3261; MGC72094; NEM1; OK/SW-cl.5; OTTHUMP00000034019; OTTHUMP00000034171; OTTHUMP00000034172; TM 5; TM-5; TM3; TM30; TM30nm; TM5; Tm5NM; Tpm 5; TPM3; TPM3/NTRK1 FUSION GENE, INCLUDED; TPM3_HUMAN; Tpm5; TPMsk3; TRK; TRK ONCOGENE, INCLUDED; Trop 5; Tropomyosin 3; Tropomyosin 3 gamma; Tropomyosin 5; Tropomyosin alpha 3 chain; Tropomyosin alpha-3 chain; Tropomyosin gamma; Tropomyosin-3; Tropomyosin-5.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  細胞生物  信號轉導  結合蛋白  細胞骨架  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, )
產品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 33kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Tropomyosin 3
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產品介紹 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]. 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 adulthood. 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 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.

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.
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