Rabbit HADHA Polyclonal Antibody | anti-HADHA antibody

HADHA Antibody

Cat. Num. AAA9403276

• Gene Names: HADHA; GBP; ECHA; HADH; LCEH; MTPA; LCHAD; TP-ALPHA
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot, Immunohistochemistry, Immunofluorescence
• Purity: Antibodies were purified by affinity purification using immunogen.
• Synonyms: HADHA; Polyclonal Antibody; HADHA Antibody; GBP; ECHA; HADH; LCEH; MTPA; anti-HADHA antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Specificity: The antibody detects endogenous level of total HADHA protein.
• Purity/Purification: Antibodies were purified by affinity purification using immunogen.
• Form/Format: Supplied at 1.0mg/mL in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
• Concentration: 1.0 mg/ml (varies by lot)
• Sequence Length: 763

• Applicable Applications for anti-HADHA antibody: Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)
• Application Notes: Western blotting: 1:500 - 1:2000; Immunohistochemistry: 1:50 - 1:200; Immunofluorescence: 1:50 - 1:200
• Immunogen Type: Recombinant Protein
• Immunogen Description: Recombinant protein of human HADHA.
• Target Name: HADHA
• Preparation and Storage: Store at -20 degree C

Western Blot (WB)

(Western blot analysis of extracts of various cell lines, using HADHA antibody.)

Immunohistochemistry (IHC)

(Immunohistochemical analysis of paraffin-embedded human thyroid cancer using HADHA antibody at dilution of 1:200 (400x lens).)

Immunofluorescence (IF)

(Immunofluorescence analysis of U2OS cell using HADHA antibody. Blue: DAPI for nuclear staining.)

Related Product Information for anti-HADHA antibody

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.

Product Categories/Family for anti-HADHA antibody

Total protein Ab

NCBI and Uniprot Product Information

• NCBI GI #: 20127408
• NCBI GeneID: 3030
• NCBI Accession #: NP_000173.2
• NCBI GenBank Nucleotide #: NM_000182.4
• UniProt Accession #: P40939; Q16679; Q53T69; Q53TA2; Q96GT7; Q9UQC5; B2R7L4; B4DYP2
• Molecular Weight: 28,367 Da
• NCBI Official Full Name: trifunctional enzyme subunit alpha, mitochondrial
• NCBI Official Synonym Full Names: hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit
• NCBI Official Symbol: HADHA
• NCBI Official Synonym Symbols: GBP; ECHA; HADH; LCEH; MTPA; LCHAD; TP-ALPHA
• NCBI Protein Information: trifunctional enzyme subunit alpha, mitochondrial
• UniProt Protein Name: Trifunctional enzyme subunit alpha, mitochondrial
• Protein Family: Trifunctional enzyme
• UniProt Gene Name: HADHA
• UniProt Synonym Gene Names: HADH
• UniProt Entry Name: ECHA_HUMAN

NCBI Description

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]

Uniprot Description

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

Chromosomal Location of Human Ortholog: 2p23

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

Molecular Function: protein binding; acetyl-CoA C-acetyltransferase activity; acyl-CoA binding; enoyl-CoA hydratase activity; long-chain-3-hydroxyacyl-CoA dehydrogenase activity; protein complex binding; long-chain-enoyl-CoA hydratase activity; NAD binding; 3-hydroxyacyl-CoA dehydrogenase activity

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

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