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HADHA: Difference between revisions

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== Function ==
 
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>{{cite journal|last1=Carpenter|first1=K|last2=Pollitt|first2=RJ|last3=Middleton|first3=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|date=16 March 1992|volume=183|issue=2|pages=443-8|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>
 
 
== Clinical significance ==