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anti-HADH antibody :: Rabbit anti-Human, Mouse HADH Polyclonal Antibody

Scan QR to view Datasheet Catalog #    MBS7045286
Unit / Price
0.05 mL  /  $120 +1 FREE 8GB USB
0.1 mL  /  $160 +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   

HADH, Polyclonal Antibody

★Popular Item★
 Also Known As   

HADH Antibody

 Product Synonym Names    Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial; HCDH; Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase; Short-chain 3-hydroxyacyl-CoA dehydrogenase; HADH; HAD; HADHSC; SCHAD
 Product Gene Name   

anti-HADH antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 OMIM    231530
 3D Structure    ModBase 3D Structure for Q16836
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 Clonality    Polyclonal
 Isotype    IgG
 Host    Rabbit
 Species Reactivity    Human, Mouse
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 Purity/Purification    Antigen Affinity Purified
 Form/Format    Liquid
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 Species    Human
 Immunogen    Recombinant human Hydroxyacyl-coenzyme A dehydrogenase, mitochondrial protein (1-314AA)
 Conjugate    Non-conjugated
 Buffer    PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
 Santa Cruz Alternative    Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-74650 / sc-74651 / sc-74653 / sc-292196
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 Preparation and Storage    Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.
 ISO Certification    Manufactured in an ISO 9001:2008 Certified Laboratory.
 Other Notes    Small volumes of anti-HADH antibody 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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Related Product Information for anti-HADH antibody

   Plays an essential role in the mitochondrial beta-oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA.
 Applications Tested/Suitable for anti-HADH antibody   

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)

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 Western Blot (WB) of anti-HADH antibody    Western blot
All lanes: HADH antibody at 1.21 ug/ml
Lane 1: Mouse heart tissue
Lane 2: Mouse liver tissue
Lane 3: Mouse kidney tissue
Secondary
Goat polyclonal to rabbit IgG at 1/10000 dilution
Predicted band size: 35,43,37 kDa
Observed band size: 34 kDa

anti-HADH antibody Western Blot (WB) (WB) image
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 Immunohistochemistry (IHC) of anti-HADH antibody    Immunohistochemistry of paraffin-embedded human kidney tissue using MBS7045286 at dilution 1:100
anti-HADH antibody Immunohistochemistry (IHC) (IHC) image
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 Immunohistochemistry (IHC) of anti-HADH antibody    Immunohistochemistry of paraffin-embedded human heart tissue using MBS7045286 at dilution 1:100
anti-HADH antibody Immunohistochemistry (IHC) (IHC) image
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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 #    296179427
 NCBI GeneID    3033
 NCBI Accession #    NP_001171634.2 [Other Products]
 NCBI GenBank Nucleotide #    NM_001184705.2 [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    36,051 Da
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 NCBI Official Full Name    hydroxyacyl-coenzyme A dehydrogenase, mitochondrial isoform 1
 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
 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 - lysine degradation; EC 1.1.1.35; Amino Acid Metabolism - tryptophan; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Lipid Metabolism - fatty acid; Oxidoreductase; Lipid Metabolism - fatty acid elongation in mitochondria; Carbohydrate Metabolism - butanoate

Chromosomal Location of Human Ortholog: 4q22-q26

Cellular Component: cytoplasm; mitochondrial matrix; mitochondrion; nucleoplasm

Molecular Function: 3-hydroxyacyl-CoA dehydrogenase activity

Biological Process: fatty acid beta-oxidation

Disease: 3-hydroxyacyl-coa Dehydrogenase Deficiency; Hyperinsulinemic Hypoglycemia, Familial, 4
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 Research Articles on HADH    1. We present clinical and laboratory findings together with the long-term clinical course of a case with a deep intronic HADH splicing mutation (c.636+471G>T) causing neonatal-onset hyperinsulinemic hypoglycemia with mild progression
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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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Pathways associated with anti-HADH antibodyDiseases associated with anti-HADH antibody
 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
 Disease Name  Pubmed Publications
 Obesity Antibodies  >13 publications with HADH and Obesity
 Disease Models, Animal Antibodies  >7 publications with HADH and Disease Models, Animal
 Fatty Liver Antibodies  >7 publications with HADH and Fatty Liver
 Cardiovascular Diseases Antibodies  >6 publications with HADH and Cardiovascular Diseases
 Weight Loss Antibodies  >6 publications with HADH and Weight Loss
 3-Hydroxyacyl-CoA Dehydrogenase Deficiency Antibodies  >5 publications with HADH and 3-Hydroxyacyl-CoA Dehydrogenase Deficiency
 Heart Diseases Antibodies  >4 publications with HADH and Heart Diseases
 Hyperplasia Antibodies  >3 publications with HADH and Hyperplasia
 Nervous System Diseases Antibodies  >3 publications with HADH and Nervous System Diseases
 Inflammation Antibodies  >2 publications with HADH and Inflammation
Organs/Tissues associated with anti-HADH antibody
 Organ/Tissue Name  Pubmed Publications
 Muscle Antibodies  >61 publications with HADH and Muscle
 Blood Antibodies  >25 publications with HADH and Blood
 Liver Antibodies  >15 publications with HADH and Liver
 Brain Antibodies  >14 publications with HADH and Brain
 Pancreas Antibodies  >10 publications with HADH and Pancreas
 Heart Antibodies  >9 publications with HADH and Heart
 Adipose Tissue Antibodies  >9 publications with HADH and Adipose Tissue
 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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