The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Blocking - Blocking peptide for Anti-HADHB antibody (ab10093)
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Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
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
パスウェイLipid metabolism; fatty acid beta-oxidation.
関連疾患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.