hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenz

Human Trifunctional enzyme subunit alpha; mitochondrial (HADHA) ELISA kit; ECHA; GBP; HADH; LCHAD; MGC1728; MTPA; TP-ALPHA; 3-ketoacyl-Coenzyme A (CoA) thiolase; alpha subunit; 3-oxoacyl-CoA thiolase; 78 kDa gastrin-binding protein; mitochondrial long-chain 2-enoyl-Coenzyme A; hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein); alpha subunit

For Research Use Only. Not for use in diagnostic procedures.

No significant cross-reactivity or interference between this analyte and analogues is observed.

Store all reagents at 2-8 degree C

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Small volumes of HADHA elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS9342072 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit (HADHA) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing HADHA. The ELISA analytical biochemical technique of the MBS9342072 kit is based on HADHA antibody-HADHA antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect HADHA antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, HADHA. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

Background: This Quantitative Sandwich ELISA kit is only for in vitro research use only, not for drug, household, therapeutic or diagnostic applications! This kit is intended to be used for determination the level of HADHA (hereafter termed "analyte") in undiluted original Human body fluids, tissue homogenates, secretions or feces samples. This kit is NOT suitable for assaying non-biological sources of substances.

82,670 Da

hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), alpha subunit

trifunctional enzyme subunit alpha, mitochondrial; TP-alpha

Trifunctional enzyme subunit alpha, mitochondrial

ECHA_MOUSE

HADHA: Bifunctional subunit. Defects in HADHA are a cause of trifunctional protein deficiency (TFP deficiency). 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 enzyme activities of the TFP complex. Defects in HADHA are the cause of long-chain 3-hydroxyl- CoA dehydrogenase deficiency (LCHAD deficiency). The clinical features are very similar to TFP deficiency. Biochemically, LCHAD deficiency is characterized by reduced long- chain 3-hydroxyl-CoA dehydrogenase activity, while the other enzyme activities of the TFP complex are normal or only slightly reduced. Defects in HADHA are a cause of maternal acute fatty liver of pregnancy (AFLP). AFLP is a severe maternal illness occurring during pregnancies with affected fetuses. This disease is associated with LCHAD deficiency and characterized by sudden unexplained infant death or hypoglycemia and abnormal liver enzymes (Reye-like syndrome).

Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; Amino Acid Metabolism - lysine degradation; EC 4.2.1.17; Carbohydrate Metabolism - butanoate; Acetyltransferase; Lipid Metabolism - fatty acid elongation in mitochondria; Lyase; EC 1.1.1.211; Amino Acid Metabolism - tryptophan; Lipid Metabolism - fatty acid; Other Amino Acids Metabolism - beta-alanine; Carbohydrate Metabolism - propanoate; Secondary Metabolites Metabolism - limonene and pinene degradation; Mitochondrial; Oxidoreductase; Lipid Metabolism - unsaturated fatty acid biosynthesis

Cellular Component: mitochondrion; mitochondrial inner membrane; fatty acid beta-oxidation multienzyme complex

Molecular Function: acyl-CoA binding; enoyl-CoA hydratase activity; lyase activity; oxidoreductase activity; acetyl-CoA C-acyltransferase activity; oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor; 3-hydroxyacyl-CoA dehydrogenase activity; long-chain-3-hydroxyacyl-CoA dehydrogenase activity; protein complex binding; long-chain-enoyl-CoA hydratase activity; coenzyme binding; catalytic activity; NAD binding

Biological Process: response to drug; fatty acid beta-oxidation; metabolic process; lipid metabolic process; fatty acid metabolic process; response to insulin stimulus

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