Figure 2. Biochemical bases and consequences of β-ketothiloase (BKT) deficiency. BKT plays a vital role in catabolism of isoleucine. BKT cleaves methylacetoacetyl CoA into acetyl CoA and propionyl CoA. Deficiency of BKT results in the accumulation of acetoacetate and lack of ketone bodies utilization. Fortunately, 1^st step of isoleucine catabolism (transamination step) is reported to be indirectly inhibited by dichloroacetate (DCA). DCA inhibits first step of isoleucine catabolism preventing ketone bodies formation and subsequent refractory metabolic acidosis. That may suggest a promising role of DCA in treating BKT deficiency.

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Dichloroacetate is a Novel Safe Treatment for Beta-ketothiolase Deficiency: Towards Better Therapeutic Outcomes (An Original Article)

Salah Mohamed El Sayed, Elsayed Abdelkreem, Moutasem Salih Aboonq, Sultan S. Al Thagfan, Yaser M. Alahmadi, Osama Alhadramy, Hussam Baghdadi, Mohammed Hassan, Faten M. Omran, Hytham Mahmoud Abdel-Latif, Wafaa Abdel-ziz, Azza Mahmoud Ahmed Abouelella, Amr El-Dardear, Mohamed Abdel-haleem, Elhussainy MA Elhussainy, Hassan El-Alaf, Manal Mohamed Helmy Nabo

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