Freidriech ataxia - biochemical mechanisms
Yesterday I watched two excellent presentations on Freidreich ataxia (still trying to use the proper nomenclature convention) from back in 2011: Notes: 5:44 frataxin is essential for muticellular eukaryotes (but not single celled organisms) frataxin is needed for the production of iron-sulfur clusters (aconitase is an important TCA enzyme that includes an iron-sulfur cluster that is particularly vulnerable to oxidation) 13:07 Indeed! & also part of the urea cycle 18:35 Reduction in frataxin leads to iron accumulation in the mitochondrion 38:26 summary of therapeutic approaches in relation to hypothetical vicious cycle in FA EPO - erythropoietin increases expression of genes related to iron utilization (lactoferrin and Wim Hof method breathing increase EPO) HDAC inhibitors increase expression of frataxin (butyrate / tributyrin are HDACi) pioglitozone increases mitochondrial biogenesis (PQQ & urolithin A do the same) I think neuroinflammation should be included as part