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HADH recombinant protein :: HADHSC Recombinant Protein

Scan QR to view Datasheet Catalog #    MBS613100 HADH recombinant protein
Unit / Price
0.05 mg  /  $540 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

HADHSC, Recombinant Protein

 Also Known As   

HADHSC, Recombinant, Human (L-3- Hydroxyacyl Coenzyme A Dehydrogenase, Short Chain)

 Product Synonym Names    Anti -HADHSC, Recombinant, Human (L-3- Hydroxyacyl Coenzyme A Dehydrogenase, Short Chain)
 Product Gene Name   

HADH recombinant protein

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Chromosome Location    Chromosome: 4; NC_000004.11 (108910870..108956331). Location: 4q22-q26
 OMIM    231530
 3D Structure    ModBase 3D Structure for Q16836
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 Clonality    Polyclonal
 Isotype    IgY
 Host    Chicken
 Species Reactivity    Human
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 Specificity    Recognizes human HADHSC.
 Purity/Purification    Affinity Purified
Purified by immunoaffinity chromatography.
 Form/Format    Supplied as a liquid in PBS, pH 7.2. No preservative added. Glycerol free.
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 Immunogen    Synthetic peptide corresponding to aa57-314
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 Preparation and Storage    May be stored at 4 degree C for short-term only. For long-term storage, store at -20 degree C. Aliquots are stable for at least 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
 Other Notes    Small volumes of HADH recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Product Description specifically for HADH recombinant protein

   Short-chain L-3-hydroxyacyl-CoA dehydrogenase (SCHAD; EC1.1.1.35) plays an essential role in the mitochondrial beta-oxidation of short chain fatty acids. It catalyzes the reversible dehydrogenation of 3-hydroxyacyl-CoAs to their corresponding 3-ketoacyl-CoAs with concomitant reduction of NAD to NADH and exerts it highest activity toward 3-hydroxybutyryl-CoA.
 Product Categories/Family for HADH recombinant protein    Antibodies; Abs to Coenzyme A
 Applications Tested/Suitable for HADH recombinant protein   

ELISA (EL/EIA), Western Blot (WB)

 Application Notes for HADH recombinant protein    Suitable for use in ELISA and Western Blot.
Dilution: Western Blot: 1:1000-1:2000
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NCBI/Uniprot data below describe general gene information for HADH. It may not necessarily be applicable to this product.
 NCBI GI #    296179429
 NCBI GeneID    3033
 NCBI Accession #    NP_005318.3 [Other Products]
 NCBI GenBank Nucleotide #    NM_005327.4 [Other Products]
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 UniProt Primary Accession #    Q16836 [Other Products]
 UniProt Secondary Accession #    O00324; O00397; O00753; Q4W5B4; J3KQ17 [Other Products]
 UniProt Related Accession #    Q16836 [Other Products]
 Molecular Weight    34,294 Da [Similar Products]
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 NCBI Official Full Name    hydroxyacyl-coenzyme A dehydrogenase, mitochondrial isoform 2
 NCBI Official Synonym Full Names    hydroxyacyl-CoA dehydrogenase
 NCBI Official Symbol    HADH [Similar Products]
 NCBI Official Synonym Symbols   
HAD; HCDH; HHF4; HADH1; SCHAD; HADHSC; MSCHAD
[Similar Products]
 NCBI Protein Information    hydroxyacyl-coenzyme A dehydrogenase, mitochondrial; short-chain 3-hydroxyacyl-CoA dehydrogenase; L-3-hydroxyacyl-Coenzyme A dehydrogenase, short chain; medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase
 UniProt Protein Name    Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial
 UniProt Synonym Protein Names   
Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase; Short-chain 3-hydroxyacyl-CoA dehydrogenase
 Protein Family    Hydroxyacyl-coenzyme A dehydrogenase
 UniProt Gene Name    HADH [Similar Products]
 UniProt Synonym Gene Names    HAD; HADHSC; SCHAD; HCDH [Similar Products]
 UniProt Entry Name    HCDH_HUMAN
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 NCBI Summary for HADH    This gene is a member of the 3-hydroxyacyl-CoA dehydrogenase gene family. The encoded protein functions in the mitochondrial matrix to catalyze the oxidation of straight-chain 3-hydroxyacyl-CoAs as part of the beta-oxidation pathway. Its enzymatic activity is highest with medium-chain-length fatty acids. Mutations in this gene cause one form of familial hyperinsulinemic hypoglycemia. The human genome contains a related pseudogene of this gene on chromosome 15. [provided by RefSeq, May 2010]
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 UniProt Comments for HADH    HADHSC: Plays an essential role in the mitochondrial beta- oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA. Defects in HADH are the cause of 3-alpha-hydroxyacyl-CoA dehydrogenase deficiency (HADH deficiency). 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); 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. Belongs to the 3-hydroxyacyl-CoA dehydrogenase family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; Lipid Metabolism - fatty acid; EC 1.1.1.35; Lipid Metabolism - fatty acid elongation in mitochondria; Carbohydrate Metabolism - butanoate; Amino Acid Metabolism - lysine degradation; Amino Acid Metabolism - tryptophan; Oxidoreductase

Chromosomal Location of Human Ortholog: 4q22-q26

Cellular Component: nucleoplasm; mitochondrion; mitochondrial matrix; mitochondrial inner membrane; cytoplasm

Molecular Function: 3-hydroxyacyl-CoA dehydrogenase activity

Biological Process: response to drug; fatty acid beta-oxidation; response to activity; cellular lipid metabolic process; response to insulin stimulus; negative regulation of insulin secretion

Disease: 3-hydroxyacyl-coa Dehydrogenase Deficiency; Hyperinsulinemic Hypoglycemia, Familial, 4
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 Research Articles on HADH    1. Next-generation sequencing reveals deep intronic cryptic ABCC8 and HADH splicing founder mutations causing hyperinsulinism by pseudoexon activation.
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with HADH recombinant proteinPathways associated with HADH recombinant protein
 Reference Product  PubMed Publications
 ACAA2 recombinant protein  >2 publications with HADH and ACAA2
 HADHB recombinant protein  >1 publications with HADH and HADHB
 ACAT1 recombinant protein  >1 publications with HADH and ACAT1
 ECHS1 recombinant protein  >1 publications with HADH and ECHS1
 Products by Pathway  Pathway Diagram
 Beta Oxidation Of Butanoyl-CoA To Acetyl-CoA Pathway antibodies  Beta Oxidation Of Butanoyl-CoA To Acetyl-CoA Pathway Diagram
 Beta Oxidation Of Decanoyl-CoA To Octanoyl-CoA-CoA Pathway antibodies  Beta Oxidation Of Decanoyl-CoA To Octanoyl-CoA-CoA Pathway Diagram
 Beta Oxidation Of Hexanoyl-CoA To Butanoyl-CoA Pathway antibodies  Beta Oxidation Of Hexanoyl-CoA To Butanoyl-CoA Pathway Diagram
 Beta Oxidation Of Lauroyl-CoA To Decanoyl-CoA-CoA Pathway antibodies  Beta Oxidation Of Lauroyl-CoA To Decanoyl-CoA-CoA Pathway Diagram
 Beta Oxidation Of Octanoyl-CoA To Hexanoyl-CoA Pathway antibodies  Beta Oxidation Of Octanoyl-CoA To Hexanoyl-CoA Pathway Diagram
 Butanoate Metabolism Pathway antibodies  Butanoate Metabolism Pathway Diagram
 Butanoate Metabolism Pathway antibodies  Butanoate Metabolism Pathway Diagram
 FOXA2 And FOXA3 Transcription Factor Networks Pathway antibodies  FOXA2 And FOXA3 Transcription Factor Networks Pathway Diagram
 Fatty Acid Beta Oxidation Pathway antibodies  Fatty Acid Beta Oxidation Pathway Diagram
 Fatty Acid Biosynthesis Pathway antibodies  Fatty Acid Biosynthesis Pathway Diagram
Diseases associated with HADH recombinant proteinOrgans/Tissues associated with HADH recombinant protein
 Disease Name  Pubmed Publications
 Obesity Antibodies  >14 publications with HADH and Obesity
 Weight Loss Antibodies  >7 publications with HADH and Weight Loss
 Cardiovascular Diseases Antibodies  >6 publications with HADH and Cardiovascular Diseases
 Disease Models, Animal Antibodies  >5 publications with HADH and Disease Models, Animal
 Heart Diseases Antibodies  >4 publications with HADH and Heart Diseases
 3-hydroxyacyl-coa dehydrogenase deficiency Antibodies  >4 publications with HADH and 3-hydroxyacyl-coa dehydrogenase deficiency
 Fatty Liver Antibodies  >3 publications with HADH and Fatty Liver
 Hyperplasia Antibodies  >3 publications with HADH and Hyperplasia
 Nervous System Diseases Antibodies  >3 publications with HADH and Nervous System Diseases
 Inflammation Antibodies  >1 publications with HADH and Inflammation
 Organ/Tissue Name  Pubmed Publications
 Muscle Antibodies  >56 publications with HADH and Muscle
 Blood Antibodies  >19 publications with HADH and Blood
 Brain Antibodies  >11 publications with HADH and Brain
 Liver Antibodies  >10 publications with HADH and Liver
 Adipose Tissue Antibodies  >9 publications with HADH and Adipose Tissue
 Heart Antibodies  >8 publications with HADH and Heart
 Pancreas Antibodies  >8 publications with HADH and Pancreas
 Connective Tissue Antibodies  >6 publications with HADH and Connective Tissue
 Lung Antibodies  >4 publications with HADH and Lung
 Kidney Antibodies  >4 publications with HADH and Kidney
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