概述

  • 产品名称Anti-SLC25A13抗体
    参阅全部 SLC25A13 一抗
  • 描述
    兔多克隆抗体to SLC25A13
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Human
    预测可用于: Mouse, Cow, Zebrafish
  • 免疫原

    Recombinant fragment, corresponding to a sequence within amino acids 248-505 of Human SLC25A13.

  • 阳性对照
    • 293T, A431, H1299, HeLa, HepG2, Molt4 or Raji whole cell lysate.

应用

Our Abpromise guarantee covers the use of ab96303 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
WB 1/500 - 1/3000. Predicted molecular weight: 74 kDa.

靶标

  • 功能Catalyzes the calcium-dependent exchange of cytoplasmic glutamate with mitochondrial aspartate across the mitochondrial inner membrane. May have a function in the urea cycle.
  • 组织特异性High levels in liver and low levels in kidney, pancreas, placenta, heart and brain.
  • 疾病相关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.
  • 序列相似性Belongs to the mitochondrial carrier family.
    Contains 4 EF-hand domains.
    Contains 3 Solcar repeats.
  • 细胞定位Mitochondrion inner membrane.
  • Information by UniProt
  • 数据库链接
  • 别名
    • AI785475 antibody
    • ARALAR2 antibody
    • Calcium binding mitochondrial carrier protein Aralar2 antibody
    • Calcium-binding mitochondrial carrier protein Aralar2 antibody
    • Citrin antibody
    • CMC2_HUMAN antibody
    • CTLN2 antibody
    • Ctrn antibody
    • Mitochondrial aspartate glutamate carrier 2 antibody
    • RGD1565889 antibody
    • Slc25a13 antibody
    • Solute carrier family 25 (citrin) member 13 antibody
    • Solute carrier family 25 member 13 (citrin) antibody
    • Solute carrier family 25 member 13 antibody
    see all

Anti-SLC25A13 antibody 图像

  • Anti-SLC25A13 antibody (ab96303) at 1/1000 dilution + 293T whole cell lysate at 30 µg

    Predicted band size : 74 kDa

Anti-SLC25A13 antibody (ab96303)参考文献

ab96303 has not yet been referenced specifically in any publications.

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