Rabbit anti-human Trifunctional enzyme subunit beta, mitochondrial polyclonal Antibody;Biotin conjugated

Product Type: Polyclonal antibody
Product Name: Rabbit anti-human Trifunctional enzyme subunit beta, mitochondrial polyclonal Antibody;Biotin conjugated
Alias: Acetyl-CoA acyltransferase,Beta-ketothiolase
Size: 100ug
Code: CSB-PA03035D0Rb
Species:
Immunogen: Recombinant human Trifunctional enzyme subunit beta, mitochondrial protein
Raised in: Rabbit
Species Reactivity: Human
Tested Applications: ELISA, WB, IHC
Application Notes:
Relevance: 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. Biochemica
Clonality: Polyclonal
Isotype: IgG
Purity: Caprylic Acid Ammonium Sulfate Precipitation purified
Conjugate: Biotin conjugated
Storage Buffer: Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Form: Liquid
Storage: Shipped at 4C. Upon delivery aliquot and store at -20C or -80C. Avoid repeated freeze.
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