FITC标记的肌管素相关蛋白14抗体-抗体-抗体-生物在线
上海沪震实业有限公司
FITC标记的肌管素相关蛋白14抗体

FITC标记的肌管素相关蛋白14抗体

商家询价

产品名称: FITC标记的肌管素相关蛋白14抗体

英文名称: Anti-MTMR14/FITC

产品编号: HZ-11217R-FITC

产品价格: null

产品产地: 中国/上海

品牌商标: HZbscience

更新时间: 2023-08-17T10:24:20

使用范围: ICC=1:50-200 IF=1:50-200

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 Rabbit Anti-MTMR14/FITC Conjugated antibody

FITC标记的肌管素相关蛋白14抗体

 

产品编号 bs-11217R-FITC
英文名称 Anti-MTMR14/FITC
中文名称 FITC标记的肌管素相关蛋白14抗体
别    名 C3orf29; Egg derived tyrosine phosphatase homolog; FLJ11546; FLJ22405; FLJ46453; FLJ90311; HCV NS5A transactivated protein 4 splice variant A binding protein 1; HCV NS5A-transactivated protein 4 splice variant A-binding protein 1; hJumpy; jumpy; MTMR 14; MTMR14; MTMR-14; MTMRE_HUMAN; Myotubularin related protein 14; Myotubularin-related protein 14; NS5ATP4ABP1.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 细胞生物  免疫学  激酶和磷酸酶  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, 
产品应用 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 72kDa
细胞定位 细胞膜 
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human MTMR14
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.
产品介绍 background:
Myotubularin-related protein 14 (MTMR14), also known as Jumpy, is a myotubularin-related phosphoinositol-3-phosphate (PI3P) phosphatase (1). Mutations in the MTMR14 gene have been associated with centronuclear myopathy (1). MTMR14 deficiency in mice leads to altered calcium homeostasis and muscle disorders (2). MTMR14 has also been shown to play a role in autophagy, a process that is highly regulated by phosphatidylinositides through the type III PI3K, Vps34 (3). MTMR14 was localized to autophagic isolation membranes and early autophagosomes (3). In these studies, MTMR14 inhibited autophagy and mutations of MTMR14 associated with centronuclear myopathy were also defective in autophagy inhibition. In zebrafish, MTMR14 knockdown was shown to increase the number of autophagosomes, suggesting that its activity is associated with an inhibition of autophagy (4).

Function:
Lipid phosphatase which efficiently dephosphorylates phosphatidylinositol 3-phosphate (PtdIns3P) and PtdIns(3,5)P2; inactive toward PtdIns4P, PtdIns(3,4)P2, PtdIns(4,5)P2 and PtdIns(3,4,5)P3.

Subunit:
Belongs to the protein-tyrosine phosphatase family. Non-receptor class myotubularin subfamily.

Subcellular Location:
Cytoplasm. Found in reticular structures and plasma membrane ruffles. Concentrated near the nucleus.

Tissue Specificity:
Expressed in various tissues, including heart, skeletal muscle, placenta, liver, lung, kidney and pancreas.

DISEASE:
Defects in MTMR14 may be a cause of centronuclear myopathy autosomal dominant (ADCNM) [MIM:160150]; also known as autosomal dominant myotubular myopathy. Centronuclear myopathies are congenital muscle disorders characterized by progressive muscular weakness and wasting involving mainly limb girdle, trunk, and neck muscles. It may also affect distal muscles. Weakness may be present during childhood or adolescence or may not become evident until the third decade of life. Ptosis is a frequent clinical feature. The most prominent histopathologic features include high frequency of centrally located nuclei in muscle fibers not secondary to regeneration, radial arrangement of sarcoplasmic strands around the central nuclei, and predominance and hypotrophy of type 1 fibers.

Similarity:
Belongs to the protein-tyrosine phosphatase family. Non-receptor class myotubularin subfamily. 

Database links:
UniProtKB/Swiss-Prot: Q8NCE2.2

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

肌动蛋白相关蛋白14(MTMR14),也称为神经氨酸酶,是肌原纤维蛋白相关磷酸肌醇-3-磷酸(PI3P)磷酸酶(1)。MTMR14基因突变与中心核肌病相关(1)。小鼠MTMR14缺乏导致钙稳态改变和肌肉紊乱(2)。MTMR14也被证明在自噬作用中发挥作用,这一过程是通过磷脂酰肌醇II通过III型PI3K、VPS34(3)高度调节的。MTMR14定位于自噬分离膜和早期自噬体(3)。在这些研究中,MTMR14抑制自噬,并且与中心核肌病相关的MTMR14的突变在自噬抑制方面也是有缺陷的。在斑马鱼中,MTMR14敲除显示增加自噬体的数量,表明其活性与自噬的抑制有关(4)。