HADHB (2-enoyl Coenzyme A (CoA) Hydratase beta Subunit, 3-ketoacyl-Coenzyme A thiolase, Acetyl CoA Acyltransferase, Beta Ketothiolase, HADH, Hydroxyacyl-Coenzyme A Dehydrogenase beta, Hydroxyacyl-Coenzyme A Dehydrogenase/3-ketoacyl-Coenzyme A Thiolase/eno

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.\n\nCellular Localization: Mitochondrial matrix\n\nPositive Control: Human brain and Jurkat extracts.\n\nApplications: \nSuitable for use in ELISA and Western Blot. Other applications not tested.\n\nRecommended Dilution:\nOptimal dilutions to be determined by the researcher.\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot Store at -20 degrees C. Aliquots are stable for at least 12 months at -20 degrees 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.

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SPECIFICATIONS

Catalog Number

H1500-12

Size

100ug

Applications

ELISA, WB

Hosts

Goat

Reactivities

Hum

Form

Supplied as a liquid in TBS, pH 7.2, 0.5% BSA.

P Type

Pab

Purity

Purified by immunoaffinity chromatography.

Isotype

IgG

References

Aoyama T et al. Fluorescence in situ hybridization mapping of the alpha and beta subunits (HADHA and HADHB) of human mitochondrial fatty acid beta-oxidation multienzyme complex to 2p23 and their evolution. Cytogenet Cell Genet 79:221-4 (1997). <PUBMED:9605857>

Additional Info

Species Crossreactivity: Crossreacts with Human.