Guest guest Posted May 31, 2004 Report Share Posted May 31, 2004 Laurie, thanks for the explanation, it sure helped. I do have another question as a result though. I understand complex II is where the krebs cycle is brought in ,I assume this is where the fatty acid takes place...if so would a defect in II cause a increase in the fatty acids in blood and urine? Also would this also cause a increase in the level of krebs cycle intermediates like fumurase and succinate etc....? I read in a article from a link from UMDF that a leighs like syndrome is caused by a complex II defect and it is dominant or recessive and it causes a increase in succinate and biopsy shows a partial defect in complex II with a enzymatic deficiecny of 50-75% of complex II activity. would a complex II level of 60% would be a partial defect???? Thanks again, Quote Link to comment Share on other sites More sharing options...
Guest guest Posted May 31, 2004 Report Share Posted May 31, 2004 You have got me here - I don't have a clue! This might be a good question to ask at our next MDA chat with one of the specialists. laurie > From: Mrsmurffy@... > Reply-To: > Date: Mon, 31 May 2004 14:00:28 EDT > To: > Subject: Re: Laurie/Barbara > > Laurie, > thanks for the explanation, it sure helped. > I do have another question as a result though. > I understand complex II is where the krebs cycle is brought in ,I assume this > is where the fatty acid takes place...if so would a defect in II cause a > increase in the fatty acids in blood and urine? Also would this also cause a > increase in the level of krebs cycle intermediates like fumurase and succinate > etc....? > I read in a article from a link from UMDF that a leighs like syndrome is > caused by a complex II defect and it is dominant or recessive and it causes a > increase in succinate and biopsy shows a partial defect in complex II with a > enzymatic deficiecny of 50-75% of complex II activity. > would a complex II level of 60% would be a partial defect???? > Thanks again, > > > > Quote Link to comment Share on other sites More sharing options...
Guest guest Posted May 31, 2004 Report Share Posted May 31, 2004 I second Laurie's suggestion--ask the experts! I can just add that many, if not most, people with inborn errors in fat metabolism have essentially normal serum lipid profiles. The genetic FOD defects are not diagnosed primarily by a derangement of lipids in blood because this does not occur in many cases and because those findings can be caused by many other conditions. Inborn errors in fat metabolism are diagnosed by measursing the activity of the essential enzymes that are catalysts for oxidizing fatty acids. Also--while fat metabolism takes place primarily in beta oxidation, it is true that all these metabolic cycles interact in complex ways. A defect in one cycle can secondarily upset the balance in another cycle. Not sure that helps....but it's all I can offer. :-) B > > > You have got me here - I don't have a clue! This might be a good question > to ask at our next MDA chat with one of the specialists. > > laurie > > > From: Mrsmurffy@a... > > Reply-To: > > Date: Mon, 31 May 2004 14:00:28 EDT > > To: > > Subject: Re: Laurie/Barbara > > > > Laurie, > > thanks for the explanation, it sure helped. > > I do have another question as a result though. > > I understand complex II is where the krebs cycle is brought in ,I assume this > > is where the fatty acid takes place...if so would a defect in II cause a > > increase in the fatty acids in blood and urine? Also would this also cause a > > increase in the level of krebs cycle intermediates like fumurase and succinate > > etc....? > > I read in a article from a link from UMDF that a leighs like syndrome is > > caused by a complex II defect and it is dominant or recessive and it causes a > > increase in succinate and biopsy shows a partial defect in complex II with a > > enzymatic deficiecny of 50-75% of complex II activity. > > would a complex II level of 60% would be a partial defect???? > > Thanks again, > > > > > > > > Quote Link to comment Share on other sites More sharing options...
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