HADHB (Trifunctional Enzyme Subunit beta, Mitochondrial, TP-beta, MSTP029)

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£544.00

SKU:: 029179
Reinheit:: Purified by Protein G affinity chromatography.
Lagerbedingungen:: -20°C
Hersteller:: United States Biological
Host:: Rabbit
Immunogen:: Recombinant corresponding to aa35-283 from human HADHB.
Format:: Supplied as a liquid in 0.01M PBS, pH 7.4, 0.03% Proclin 300, 50% glycerol.
Weitere Details:: 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. Applications: Suitable for use in ELISA and Immunohistochemistry. Other applications not tested. Recommended Dilution: Immunohistochemistry: 1:20-1:200 Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.