HADHA, Monoclonal Antibody

78 kDa gastrin binding protein; ECHA; HADH; HADHA; LCHAD; MTPA; TP ALPHA

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

Monoclonal

IgG1

Mouse

Protein G purification

1mg/mL (lot specific)

Store at -20 degree C. Avoid freeze/ thaw cycles.

Manufactured in an ISO 9001:2015 Certified Laboratory.

Small volumes of anti-HADHA antibody 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.

HADHA (Trifunctional enzyme subunit alpha, mitochondrial) is also named as HADH,78 kDa gastrin-binding protein.It belongs to the enoyl-CoA hydratase/isomerase family in the N-terminal section and the 3-hydroxyacyl-CoA dehydrogenase family in the central section.It harbors the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities.Defects in HADHA are a cause of trifunctional protein deficiency (TFP deficiency) and long-chain 3-hydroxyl-CoA dehydrogenase deficiency (LCHAD deficiency) and maternal acute fatty liver of pregnancy (AFLP).

Cancer

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)

Western Blot: 1:500-1:5000
Immunohistochemistry:1:20-1:200

Western Blot analysis of HEK293 cells using HADHA Monoclonal Antibody at dilution of 1:4000

28,367 Da

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

trifunctional enzyme subunit alpha, mitochondrial

Trifunctional enzyme subunit alpha, mitochondrial

HADH  [Similar Products]

ECHA_HUMAN

This gene encodes the alpha subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the alpha subunit catalyzing the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities. Mutations in this gene result in trifunctional protein deficiency or LCHAD deficiency. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation. [provided by RefSeq, Jul 2008]

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

Chromosomal Location of Human Ortholog: 2p23

Cellular Component: mitochondrial inner membrane; mitochondrion

Molecular Function: 3-hydroxyacyl-CoA dehydrogenase activity; acetyl-CoA C-acetyltransferase activity; protein binding

Biological Process: fatty acid beta-oxidation

Disease: Long-chain 3-hydroxyacyl-coa Dehydrogenase Deficiency; Trifunctional Protein Deficiency

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