Rabbit HADHB Polyclonal Antibody | anti-HADHB antibody

HADHB antibody

Cat. Num. AAA9408199

• Gene Names: HADHB; ECHB; MTPB; MSTP029; TP-BETA
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot, Immunofluorescence
• Purity: Antibodies were purified by affinity purification using immunogen.
• Synonyms: HADHB; Polyclonal Antibody; HADHB antibody; ECHB; MTPB; MSTP029; TP-BETA; anti-HADHB antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Specificity: The antibody detects endogenous level of total HADHB antibody.
• 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: 474

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

Western Blot (WB)

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

Immunofluorescence (IF)

(Immunofluorescence analysis of A549 cell using HADHB antibody. Blue: DAPI for nuclear staining.)

Related Product Information for anti-HADHB antibody

This gene encodes the beta 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 beta subunit catalyzing the 3-ketoacyl-CoA thiolase activity. The encoded protein can also bind RNA and decreases the stability of some mRNAs. 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. Mutations in this gene result in trifunctional protein deficiency. Alternatively spliced transcript variants encoding different isoforms have been described.

Product Categories/Family for anti-HADHB antibody

Total protein Ab

NCBI and Uniprot Product Information

• NCBI GI #: 4504327
• NCBI GeneID: 3032
• NCBI Accession #: NP_000174.1
• NCBI GenBank Nucleotide #: NM_000183.2
• UniProt Accession #: P55084; O14969; Q53TA6; Q96C77; Q9H3F5; Q9T2V8; B2RB16; B4E2W0
• Molecular Weight: 48,880 Da
• NCBI Official Full Name: trifunctional enzyme subunit beta, mitochondrial isoform 1
• NCBI Official Synonym Full Names: hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit
• NCBI Official Symbol: HADHB
• NCBI Official Synonym Symbols: ECHB; MTPB; MSTP029; TP-BETA
• NCBI Protein Information: trifunctional enzyme subunit beta, mitochondrial
• UniProt Protein Name: Trifunctional enzyme subunit beta, mitochondrial
• Protein Family: Trifunctional enzyme
• UniProt Gene Name: HADHB
• UniProt Entry Name: ECHB_HUMAN

NCBI Description

This gene encodes the beta 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 beta subunit catalyzing the 3-ketoacyl-CoA thiolase activity. The encoded protein can also bind RNA and decreases the stability of some mRNAs. 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. Mutations in this gene result in trifunctional protein deficiency. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Jul 2013]

Uniprot Description

HADHB: Defects in HADHB 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 three enzyme activities of the TFP complex. Belongs to the thiolase family.

Protein type: EC 2.3.1.16; Lipid Metabolism - fatty acid elongation in mitochondria; Amino Acid Metabolism - valine, leucine and isoleucine degradation; Transferase; Lipid Metabolism - fatty acid

Chromosomal Location of Human Ortholog: 2p23

Cellular Component: mitochondrial envelope; mitochondrial outer membrane; mitochondrion; endoplasmic reticulum; mitochondrial inner membrane; fatty acid beta-oxidation multienzyme complex

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

Biological Process: fatty acid beta-oxidation; phospholipid metabolic process; glycerophospholipid biosynthetic process; cellular lipid metabolic process

Disease: Trifunctional Protein Deficiency