线粒体二羧酸载体蛋白20抗体elisa实验

英文名称  Anti-SLC25A20 

中文名称  线粒体二羧酸载体蛋白20抗体 

线粒体二羧酸载体蛋白20抗体别    名  CAC; CACT; Carnitine/acylcarnitine translocase; Solute carrier family 25 member 20; MCAT_HUMAN. 

浓    度  1mg/1ml 

规 格  0.1ml/100μg  0.2ml/200μg 

抗体来源  Rabbit  

克隆类型  polyclonal 

交叉反应  Human, Mouse, Rat, Cow, Horse, Rabbit   

产品类型  一抗    

研究领域  肿瘤 细胞生物 免疫学 染色质和核信号 信号转导  

蛋白分子量  predicted molecular weight: 33kDa 

性    状  Lyophilized or Liquid 

免 疫 原  KLH conjugated synthetic peptide derived from human SLC25A20 

亚    型  IgG 

纯化方法  affinity purified by Protein A 

储 存 液  0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide 

产品应用   WB=1:100-500  ELISA=1:500-1000  IP=1:20-100  IHC-P=1:100-500  IHC-F=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.  

线粒体二羧酸载体蛋白20抗体保存条件  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. 

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

产品介绍 SLC25A20 is one of several closely related mitochondrial membrane carrier proteins that shuttle substrates between cytosol and the intramitochondrial matrix space. It mediates the transport of acylcarnitines into the mitochondrial matrix for their oxidation by the mitochondrial fatty acid oxidation pathway. Mutations in this gene are associated with carnitine acylcarnitine translocase deficiency, which can cause a variety of pathological conditions such as hypoglycemia, cardiac arrest, hepatomegaly, hepatic dysfunction and muscle weakness, and is usually lethal in new born and infants.

Function : Mediates the transport of acylcarnitines of different length across the mitochondrial inner membrane from the cytosol to the mitochondrial matrix for their oxidation by the mitochondrial fatty acid-oxidation pathway.

Subcellular Location : Mitochondrion inner membrane; Multi-pass membrane protein.

DISEASE : Carnitine-acylcarnitine translocase deficiency (CACT deficiency) [MIM:212138]: A rare long-chain fatty acid oxidation disorder. Metabolic consequences include hypoketotic hypoglycemia under fasting conditions, hyperammonemia, elevated creatine kinase and transaminases, dicarboxylic aciduria, very low free carnitine and abnormal acylcarnitine profile with marked elevation of the long-chain acylcarnitines. Clinical features include neurologic abnormalities, cardiomyopathy, arrhythmias, skeletal muscle damage, liver dysfunction and episodes of life-threatening coma, which eventually lead to death. Most patients become symptomatic in 线粒体二羧酸载体蛋白20抗体the neonatal period with a rapidly progressive deterioration and a high mortality rate. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity : Belongs to the mitochondrial carrier (TC 2.A.29) family. 

Contains 3 Solcar repeats. 

Database links : UniProtKB/Swiss-Prot: O43772.1