Anti- HADHB Antibody

Product name : Anti- HADHB Antibody

Catalog number : GEN9127178

Supplier : MBS Polyclonals

Price : 304 EUR

Size : 50ug

More about:

Concept : Scope note: Identification of proteins or peptides that have been electrophoretically separated by blot transferring from the electrophoresis gel to strips of nitrocellulose paper, followed by labeling with antibody probes.

Discovery year : 1994-12-16

Entrez Gene : hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit  (HADHB)

Entrez gene record : 3032

Gene : HADHB

Gene ontology - Biological process : fatty acid beta-oxidation cardiolipin acyl-chain remodeling

Gene ontology - Cellular component : extracellular exosome endoplasmic reticulum mitochondrion mitochondrial outer membrane mitochondrial inner membrane mitochondrial nucleoid mitochondrial envelope

Gene ontology - Molecular function : protein binding RNA binding 3-hydroxyacyl-CoA dehydrogenase activity enoyl-CoA hydratase activity acetyl-CoA C-acyltransferase activity long-chain-3-hydroxyacyl-CoA dehydrogenase activity

Havana BLAST/BLAT : OTTHUMG00000096978

Identity : 4803

InterPro : Thiolase, active site Thiolase  (ACAT2) Thiolase-like Thiolase, conserved site Thiolase, N-terminal Thiolase, C-terminal Thiolase, acyl-enzyme intermediate active site

Kegg : Fatty acid degradation - Homo sapiens (human) Valine, leucine and isoleucine degradation - Homo sapiens (human) Fatty acid elongation - Homo sapiens (human)

Locus : 2p23.3

Long gene name : hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit beta

Name : Blotting, Western

PubMed : HADHB trifunctional enzyme, human renin, and poly(C)-binding protein are novel renin mRNA-binding proteins that target a cis-element in the 3'-UTR of renin mRNA and regulate renin production Both alpha- and beta-subunit mutations result in TFP complex instability, demonstrating that the mechanism of disease is the same in alpha- or beta-mutation-derived disease and explaining the biochemical and clinical similarities. The common disease causing mutation of G1528C in MTP gene in caucasian in probably not a common mutation in Chinese Han people in Beijing. The present findings showed that all missense mutations in HADHB were disease-causing. Results emphasize the value of cDNA analysis in the characterization of HADHA and HADHB mutations and further strengthen the model of haploinsufficiency as a major pathomechanism in MTP defects. HADHB is a functional molecular target of estrogen receptor alpha in the mitochondria, and the interaction may play an important role in the estrogen-mediated lipid metabolism in animals and humans. The results demonstrated that ERbeta was indeed associated and colocalized with HADHB within mitochondria. Mutations in HADHB, which encodes the beta-subunit of mitochondrial trifunctional protein, cause infantile onset hypoparathyroidism and peripheral polyneuropathy. nonstructural protein 5 (NS5) interacted with hydroxyacyl-CoA dehydrogenase alpha and beta subunits, two components of the mitochondrial trifunctional protein (MTP) involved in LCFA beta-oxidation Exposure to bezafibrate (400 muM for 48 h) increased the abundance of HADHA and HADHB mRNAs.

Pubmed identfication : 9605857

Qualifiers : classification, economics, history, instrumentation, methods, standards, trends, utilization, veterinary, statistics & numerical data, ethics

RefSeq identity : NM_000183

Synonyms : MTPB

Synonyms gene name : hydroxyacyl-Coenzyme A dehydrogenase/3-ketoacyl-Coenzyme A thiolase/enoyl-Coenzyme A hydratase (trifunctional protein), beta subunit hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit

Synonyms name : mitochondrial trifunctional protein, beta subunit

Tree numbers : E05.196.401.143 E05.301.300.096 E05.478.566.320.200 E05.601.262 E05.601.470.320.200

WikiPathWays : Sudden Infant Death Syndrome (SIDS) Susceptibility Pathways Fatty Acid Beta Oxidation Valproic acid pathway