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  • 产品名称：anti-Hydroxyacyl-CoADehydrogenase/3-Ketoacyl-CoAThiolase/enoyl-CoAHydratase(Tri

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  • 产品厂商：SXBIO
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简单介绍：
anti-Hydroxyacyl-CoADehydrogenase/3-Ketoacyl-CoAThiolase/enoyl-CoAHydratase(TrifunctionalProtein),betaSubunit(HADHB)antibody
详情介绍：
Immunogen Recombinant human Trifunctional enzyme subunit beta, mitochondrial protein
Isotype IgG
Purification Caprylic Acid Ammonium Sulfate Precipitation
Alternative Name Trifunctional enzyme subunit beta, mitochondrial (HADHB Antibody Abstract)
Background 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.
UniProt P55084
Pathways Monocarboxylic Acid Catabolic Process
Application Notes Optimal working concentration should be determinde by the investigator.
Restrictions For Research Use only
Format Liquid
Buffer PBS
Handling Advice Avoid repeated freeze.
Storage -20 °C/-80 °C
Storage Comment Upon delivery aliquot and store at -20 °C or -80 °C.