线粒体内钙结合天冬氨酸/谷氨酸载体蛋白抗体

英文名称    SLC25A13    

中文名称    线粒体内钙结合天冬氨酸/谷氨酸载体蛋白抗体    

别    名    ARALAR2; Calcium binding mitochondrial carrier protein Aralar2; Citrin; CTLN2; Ctrn; Mitochondrial aspartate glutamate carrier 2; RGD1565889; Solute carrier family 25 (citrin) member 13; Solute carrier family 25 member 13 (citrin); Solute carrier family 25 member 13; AI785475; CMC2_HUMAN.    

线粒体内钙结合天冬氨酸/谷氨酸载体蛋白抗体      

说 明 书    0.1ml  0.2ml      

研究领域    肿瘤  细胞生物  免疫学  神经生物学  结合蛋白  线粒体      

抗体来源    Rabbit    

克隆类型    Polyclonal    

交叉反应    Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse,     

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.    

分 子 量    74kDa    

细胞定位    细胞浆 细胞膜 线粒体    

性    状    Lyophilized or Liquid    

浓    度    1mg/1ml    

免 疫 原    KLH conjugated synthetic peptide derived from human SLC25A13    

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    

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.    

线粒体内钙结合天冬氨酸/谷氨酸载体蛋白抗体产品介绍    background:
SLC25A13 is a member of the mitochondrial carrier family. It contains four EF-hand Ca(2+) binding motifs in the N-terminal domain, and localizes to mitochondria. It catalyzes the exchange of aspartate for glutamate and a proton across the inner mitochondrial membrane, and is stimulated by calcium on the external side of the inner mitochondrial membrane. Mutations in the SLC25A13 gene result in citrullinemia, type II. Multiple transcript variants encoding different isoforms have been found for this gene.

Function:
Catalyzes the calcium-dependent exchange of cytoplasmic glutamate with mitochondrial aspartate across the mitochondrial inner membrane. May have a function in the urea cycle. 

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

Tissue Specificity:
High levels in liver and low levels in kidney, pancreas, placenta, heart and brain. 

DISEASE:
Defects in SLC25A13 are the cause of citrullinemia type 2 (CTLN2) [MIM:603471]. Citrullinemia belongs to the urea cycle disorders. It is an autosomal recessive disease characterized primarily by elevated serum and urine citrulline levels. Ammonia intoxication is another manifestation. CTLN2 is characterized by neuropsychiatric symptoms including abnormal behaviors, loss of memory, seizures and coma. Death can result from brain edema. Onset is sudden and usually between the ages of 20 and 50 years. 
Defects in SLC25A13 are the cause of neonatal intrahepatic cholestasis due to citrin deficiency (NICCD) [MIM:605814]. NICCD is a form of citrullinemia type 2 with neonatal onset. NICCD is characterized by suppression of the bile flow, hepatic fibrosis, low birth weight, growth retardation, hypoproteinemia, variable liver dysfunction. NICCD is generally not severe and symptoms disappear by one year of age with an appropriate diet. Years or even decades later, however, some individuals develop the characteristic features of citrullinemia type 2 with neuropsychiatric symptoms. 

Similarity:
Belongs to the mitochondrial carrier family. 
Contains 4 EF-hand domains. 
Contains 3 Solcar repeats. 

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

线粒体内钙结合天冬氨酸/谷氨酸载体蛋白抗体

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