Overview

  • Product nameAnti-HADHSC antibody
    See all HADHSC primary antibodies
  • Description
    Goat polyclonal to HADHSC
  • Tested applicationsSuitable for: WBmore details
  • Species reactivity
    Reacts with: Human
    Predicted to work with: Pig, Caenorhabditis elegans
  • Immunogen

    Synthetic peptide:

    C-YERGDASKEDID

    , corresponding to internal sequence amino acids 234-245 of Human HADHSC

  • Positive control
    • Human heart, muscle and kidney lysates

Properties

Associated products

Applications

Our Abpromise guarantee covers the use of ab65948 in the following tested applications.

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

Application Abreviews Notes
WB
  • Application notesWB: Use at a concentration of 0.01 - 0.03 µg/ml. Detects a band of approximately 34 kDa (predicted molecular weight: 34 kDa).

    Peptide ELISA: antibody detection limit dilution 1:64,000.

    Not yet tested in other applications.
    Optimal dilutions/concentrations should be determined by the end user.
  • Target

    • FunctionPlays an essential role in the mitochondrial beta-oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA.
    • Tissue specificityExpressed in liver, kidney, pancreas, heart and skeletal muscle.
    • PathwayLipid metabolism; fatty acid beta-oxidation.
    • Involvement in diseaseDefects 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.
    • Sequence similaritiesBelongs to the 3-hydroxyacyl-CoA dehydrogenase family.
    • Cellular localizationMitochondrion matrix.
    • Information by UniProt
    • Database links
    • Alternative names
      • 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 images

    • Anti-HADHSC antibody (ab65948) at 0.02 µg/ml + Human kidney lysate at 35 µg

      Predicted band size : 34 kDa
      Observed band size : 34 kDa

    References for Anti-HADHSC antibody (ab65948)

    ab65948 has not yet been referenced specifically in any publications.

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