The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Protein concentration is above or equal to 0.05 mg/ml.
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Preparation and Storage
Stability and Storage
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
pH: 8.00 Constituents: 0.31% Glutathione, 0.79% Tris HCl
2 enoyl Coenzyme A (CoA) hydratase beta subunit
3 ketoacyl Coenzyme A (CoA) thiolase of mitochondrial trifunctional protein beta subunit
3 ketoacyl Coenzyme A thiolase
3 ketoacyl Coenzyme A thiolase of mitochondrial trifunctional protein beta subunit
Acetyl CoA acyltransferase
Hydroxyacyl CoA dehydrogenase/3 ketoacyl CoA thiolase/enoyl CoA hydratase (trifunctional protein), beta subunit
Hydroxyacyl Coenzyme A (CoA) dehydrogenase beta subunit
Hydroxyacyl Coenzyme A dehydrogenase
Hydroxyacyl Coenzyme A dehydrogenase beta subunit
Hydroxyacyl Coenzyme A dehydrogenase/3 ketoacyl Coenzyme A thiolase/enoyl Coenzyme A hydratase (trifunctional protein) beta subunit
Mitochondrial trifunctional enzyme beta subunit
Mitochondrial trifunctional protein beta subunit
Trifunctional enzyme subunit beta
Trifunctional enzyme subunit beta mitochondrial
Lipid metabolism; fatty acid beta-oxidation.
Involvement in disease
Defects 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.