概述

  • 产品名称
  • 描述
    山羊多克隆抗体to CPT2
  • 经测试应用
    适用于: WBmore details
  • 种属反应性
    与反应: Human
  • 免疫原

    Synthetic peptide:

    C-TDSTVTVQKLNFE

    , corresponding to internal sequence amino acids 406-418 of Human CPT2 (NP_000089.1).

  • 阳性对照
    • Human liver lysate

性能

应用

Our Abpromise guarantee covers the use of ab99188 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 Use a concentration of 1 - 3 µg/ml. Detects a band of approximately 70 , 37 kDa (predicted molecular weight: 74 kDa).

靶标

  • 通路
    Lipid metabolism; fatty acid beta-oxidation.
  • 疾病相关
    Defects in CPT2 are the cause of carnitine palmitoyltransferase 2 deficiency (CPT2D) [MIM:255110, 600649]; also known as CPT-II deficiency or CPT2 deficiency. CPT2D is an autosomal recessive disorder characterized by recurrent myoglobinuria, episodes of muscle pain, stiffness, and rhabdomyolysis. These symptoms are triggered by prolonged exercise, fasting or viral infection and patients are usually young adults. In addition to this classical, late-onset, muscular type, a hepatic or hepatocardiomuscular form has been reported in infants. Clinical pictures in these children or neonates include hypoketotic hypoglycemia, liver dysfunction, cardiomyopathy and sudden death.
    Defects in CPT2 are the cause of carnitine palmitoyltransferase 2 deficiency, lethal neonatal (CPT2D-LN) [MIM:608836]; also known as lethal neonatal CPT-II deficiency. It is a lethal neonatal form of CPT2D. This rarely presentation is antenatal with cerebral periventricular cysts and cystic dysplastic kidneys. The clinical variability of the disease is likely attributed to the variable residual enzymatic activity.
  • 序列相似性
    Belongs to the carnitine/choline acetyltransferase family.
  • 细胞定位
    Mitochondrion inner membrane.
  • Information by UniProt
  • 数据库链接
  • 别名
    • Carnitine O palmitoyltransferase 2 antibody
    • Carnitine O palmitoyltransferase 2 mitochondrial antibody
    • Carnitine O-palmitoyltransferase 2 antibody
    • Carnitine palmitoyltransferase 2 antibody
    • Carnitine palmitoyltransferase II antibody
    • CPT 1 antibody
    • CPT 2 antibody
    • CPT II antibody
    • CPT1 antibody
    • CPT2 antibody
    • CPT2_HUMAN antibody
    • CPTASE antibody
    • CPTII antibody
    • IIAE4 antibody
    • mitochondrial antibody
    see all

图片

  • Anti-CPT2 antibody (ab99188) at 0.5 µg/ml + Human liver lysate (in RIPA buffer) at 35 µg
    Developed using the ECL technique

    Predicted band size : 74 kDa

实验方案

文献

ab99188 has not yet been referenced specifically in any publications.

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