HADHA: Difference between revisions

'''HydroxyacylTrifunctional enzyme subunit alpha, mitochondrial''' also known as '''hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit''' is a [[protein]] that in humans is encoded by the HADHA [[gene]]. 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<ref name="entrez">{{cite web | title = Entrez Gene: Hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit | url = http://www.ncbi.nlm.nih.gov/gene/3030 }}</ref>

'''HADHA''' is a 82.9 kDa protein composed of 763 amino acids.<ref name=COPaKB>]{{vcite2 journal | vauthors = Zong NC, Li H, Li H, Lam MP, Jimenez RC, Kim CS, Deng N, Kim AK, Choi JH, Zelaya I, Liem D, Meyer D, Odeberg J, Fang C, Lu HJ, Xu T, Weiss J, Duan H, Uhlen M, Yates JR, Apweiler R, Ge J, Hermjakob H, Ping P | title = Integration of cardiac proteome biology and medicine by a specialized knowledgebase | journal = Circulation Research | volume = 113 | issue = 9 | pages = 1043-53 | date = Oct 2013 | pmid = 23965338 | pmc = 4076475 | doi = 10.1161/CIRCRESAHA.113.301151 }}</ref><ref name="url_COPaKB">{{cite web | url = http://www.heartproteome.org/copa/ProteinInfo.aspx?QType=Protein%20ID&QValue=P40939 | work = Cardiac Organellar Protein Atlas Knowledgebase (COPaKB) | title = hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit }}</ref>

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. 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. <ref name="entrez"/>

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. <ref name="entrez"/>The enzyme converts medium- and long-chain 2-enoyl-CoA compounds into the following 3-ketoacyl-CoA when NAD is solely present, and acetyl-CoA when NAD and CoASH are present. <ref>{{vcite2 journal | vauthors = Carpenter K, Pollitt RJ, Middleton B | title = Human liver long-chain 3-hydroxyacyl-coenzyme A dehydrogenase is a multifunctional membrane-bound beta-oxidation enzyme of mitochondria | journal = Biochemical and Biophysical Research Communications | volume = 183 | issue = 2 | pages = 443-8 | date = Mar 1992 | pmid = 1550553 }}</ref> The alpha subunit catalyzes this reaction, and is attached to HADHB, which catalyzes the last step of the reaction. <ref name="BioChem">{{cite book | title = Principles of Biochemistry | chapter = Chapter 18, Mitochondrial ATP synthesis | first1 = Donald J. | last1 = Voet | first2 = Judith G. | last2 = Voet | first3 = Charlotte W. | last3 = Pratt | publisher = Wiley | year = 2010 | isbn = 978-0-470-23396-2 | page = 669 | edition = 4th | name-list-format = vanc }}</ref>