概述

  • 产品名称Anti-HADHSC抗体
    参阅全部 HADHSC 一抗
  • 描述
    兔多克隆抗体to HADHSC
  • 经测试应用适用于: WB, ELISAmore details
  • 种属反应性
    与反应: Human
    预测可用于: Mouse, Rat, Rabbit, Horse, Chicken, Guinea pig, Cow, Cat, Dog, Pig, Saccharomyces cerevisiae, Caenorhabditis elegans, Zebrafish
  • 免疫原

    Synthetic peptide, corresponding to a region within internal sequence amino acids 252-301 (AGYPMGPFEL LDYVGLDTTK FIVDGWHEMD AENPLHQPSP SLNKLVAENK) of human HADHSC (NP_005318).

  • 阳性对照
    • Fetal heart lysate.

性能

应用

Our Abpromise guarantee covers the use of ab81492 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 µg/ml. Predicted molecular weight: 35 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.
ELISA Use at an assay dependent concentration.

ELISA titre using peptide based assay, 1:312500.

靶标

  • 功能Plays an essential role in the mitochondrial beta-oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA.
  • 组织特异性Expressed in liver, kidney, pancreas, heart and skeletal muscle.
  • 通路Lipid metabolism; fatty acid beta-oxidation.
  • 疾病相关Defects in HADH are the cause of 3-alpha-hydroxyacyl-CoA dehydrogenase deficiency (HADH deficiency) [MIM:231530]. HADH deficiency is a metabolic disorder with various clinical presentations including hypoglycemia, hepatoencephalopathy, myopathy or cardiomyopathy, and in some cases sudden death.
    Defects in HADH are the cause of familial hyperinsulinemic hypoglycemia type 4 (HHF4) [MIM:609975]; also known as persistent hyperinsulinemic hypoglycemia of infancy (PHHI) or congenital hyperinsulinism. HHF is the most common cause of persistent hypoglycemia in infancy and is due to defective negative feedback regulation of insulin secretion by low glucose levels. It causes nesidioblastosis, a diffuse abnormality of the pancreas in which there is extensive, often disorganized formation of new islets. Unless early and aggressive intervention is undertaken, brain damage from recurrent episodes of hypoglycemia may occur. HHF4 should be easily recognizable by analysis of acylcarnitine species and that this disorder responds well to treatment with diazoxide. It provides the first 'experiment of nature' that links impaired fatty acid oxidation to hyperinsulinism and that provides support for the concept that a lipid signaling pathway is implicated in the control of insulin secretion.
  • 序列相似性Belongs to the 3-hydroxyacyl-CoA dehydrogenase family.
  • 细胞定位Mitochondrion matrix.
  • Information by UniProt
  • 数据库链接
  • 别名
    • 3 hydroxyacyl Coenzyme A dehydrogenase antibody
    • HAD antibody
    • HADH antibody
    • HADH1 antibody
    • HADHSC antibody
    • HADHSC, formerly antibody
    • HADSC, formerly antibody
    • HCDH antibody
    • HCDH_HUMAN antibody
    • HHF4 antibody
    • Hydroxyacyl CoA dehydrogenase antibody
    • Hydroxyacyl-coenzyme A dehydrogenase antibody
    • hydroxyacyl-coenzyme A dehydrogenase, mitochondrial antibody
    • L 3 hydroxyacyl Coenzyme A dehydrogenase short chain antibody
    • M SCHAD antibody
    • Medium and short chain L 3 hydroxyacyl coenzyme A dehydrogenase antibody
    • Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase antibody
    • MGC8392 antibody
    • mitochondrial antibody
    • MSCHAD antibody
    • OTTHUMP00000162626 antibody
    • OTTHUMP00000219688 antibody
    • SCHAD antibody
    • SCHAD, formerly antibody
    • Short chain 3 hydroxyacyl CoA dehydrogenase mitochondrial antibody
    • short chain 3-hydroxyacyl-coa dehydrogenase antibody
    • Short-chain 3-hydroxyacyl-CoA dehydrogenase antibody
    see all

Anti-HADHSC antibody 图像

  • Anti-HADHSC antibody (ab81492) at 1 µg/ml (in 5% skim milk / PBS buffer) + fetal heart lysate at 10 µg

    Secondary
    HRP conjugated anti-Rabbit IgG at 1/50000 dilution

    Predicted band size : 35 kDa
    Observed band size : 35 kDa

Anti-HADHSC antibody (ab81492)参考文献

ab81492 has not yet been referenced specifically in any publications.

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