- Datasheet
- Specific References
- Protocols
Overview
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Product nameAnti-HADHB antibody
See all HADHB primary antibodies -
DescriptionGoat polyclonal to HADHB
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Host speciesGoat
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Tested applicationsSuitable for: WBmore details
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Species reactivityReacts with: Human
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Immunogen
Synthetic peptide: TILTYPFKNLPT, corresponding to N terminal amino acids 2-13 of Human HADHB.
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Positive control
- Human brain and Jurkat extracts.
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General notes
The HADHA and HADHB genes encode the alpha and beta subunits of the mitochondrial trifunctional protein, respectively. The heterocomplex contains 4 alpha and 4 beta subunits and catalyzes 3 steps in the beta-oxidation of fatty acids, including the long-chain 3-hydroxyl-CoA dehydrogenase step. Deficiency of this complex causes sudden unexplained infant death, Reye-like syndrome, cardiomyopathy, or skeletal myopathy. Defects in HADHB are a cause of a variety of diseases such as hypoglycemia, hyperammonemia, mild liver dysfunction and 3-hydroxydicarboxylic aciduria.
Properties
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FormLiquid
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Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
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Storage bufferpH: 7.30
Preservative: 0.02% Sodium azide
Constituents: Tris buffered saline, 0.5% BSA -
Concentration information loading... -
PurityImmunogen affinity purified
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Purification notesPurified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
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Primary antibody notesThe HADHA and HADHB genes encode the alpha and beta subunits of the mitochondrial trifunctional protein, respectively. The heterocomplex contains 4 alpha and 4 beta subunits and catalyzes 3 steps in the beta-oxidation of fatty acids, including the long-chain 3-hydroxyl-CoA dehydrogenase step. Deficiency of this complex causes sudden unexplained infant death, Reye-like syndrome, cardiomyopathy, or skeletal myopathy. Defects in HADHB are a cause of a variety of diseases such as hypoglycemia, hyperammonemia, mild liver dysfunction and 3-hydroxydicarboxylic aciduria.
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ClonalityPolyclonal
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IsotypeIgG
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Research areas
Associated products
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Compatible Secondaries
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Immunizing Peptide (Blocking)
Applications
Our Abpromise guarantee covers the use of ab10093 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 | Use at an assay dependent concentration. Can be blocked with Human HADHB peptide (ab23180). |
Target
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PathwayLipid metabolism; fatty acid beta-oxidation.
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Involvement in diseaseDefects in HADHB are a cause of trifunctional protein deficiency (TFP deficiency) [MIM:609015]. The clinical manifestations are very variable and include hypoglycemia, cardiomyopathy and sudden death. Phenotypes with mainly hepatic and neuromyopathic involvement can also be distinguished. Biochemically, TFP deficiency is defined by the loss of all three enzyme activities of the TFP complex.
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Sequence similaritiesBelongs to the thiolase family.
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Cellular localizationMitochondrion.
- Information by UniProt
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Database links
- Entrez Gene: 3032 Human
- Omim: 143450 Human
- SwissProt: P55084 Human
- Unigene: 515848 Human
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Alternative names
- 2 enoyl Coenzyme A (CoA) hydratase beta subunit antibody
- 3 ketoacyl Coenzyme A (CoA) thiolase of mitochondrial trifunctional protein beta subunit antibody
- 3 ketoacyl Coenzyme A thiolase antibody
see all
Protocols
References
ab10093 has not yet been referenced specifically in any publications.
Customer reviews and Q&As
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"