Overview

  • Product name
  • Description
    Goat polyclonal to HADHSC
  • Tested applications
    Suitable for: IHC-P, WBmore details
  • Species reactivity
    Reacts with: Human
    Predicted to work with: Mouse, Rat, Horse, Chicken, Dog, Pig, Monkey, Gorilla
  • Immunogen

    Synthetic peptide corresponding to Human HADHSC aa 324-245 (internal sequence) (Cysteine residue).
    Sequence:

    YERGDASKEDID


    Database link: P00348

  • Positive control
    • Human kidney lysate. Human liver tissue.

Properties

Applications

Our Abpromise guarantee covers the use of ab195796 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
IHC-P Use a concentration of 5 µg/ml.
WB Use a concentration of 0.01 - 0.03 µg/ml. Predicted molecular weight: 34 kDa.

Target

  • Function
    Plays an essential role in the mitochondrial beta-oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA.
  • Tissue specificity
    Expressed in liver, kidney, pancreas, heart and skeletal muscle.
  • Pathway
    Lipid metabolism; fatty acid beta-oxidation.
  • Involvement in disease
    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.
  • Sequence similarities
    Belongs to the 3-hydroxyacyl-CoA dehydrogenase family.
  • Cellular localization
    Mitochondrion 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

Images

  • Anti-HADHSC antibody (ab195796) at 0.02 µg/ml + Human kidney lysate in RIPA buffer at 35 µg

    Predicted band size : 34 kDa
  • Immunohistochemical analysis of formalin-fixed, paraffin-embedded human liver tissue labeling HADHSC with ab195796 at 5 µg/mL.

References

ab195796 has not yet been referenced specifically in any publications.

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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

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