Novel approach for evaluation of mitochondrial substrate utilization in fibroblasts from patients with Friedreich’s ataxia

Ahmadian N, Dallas S, Dedkova EN. Novel approach for evaluation of mitochondrial substrate utilization in fibroblasts from patients with Friedreich’s ataxia. Biophysical Journal. 8 de febrero de 2024;123(3):523a. doi:10.1016/j.bpj.2023.11.3162

We found that FA fibroblasts had decreased utilization of alpha-ketoglutaric, succinic, fumaric and malic acids. Utilization of alpha-keto-butyric acid was not changed, however, utilization of D, L-beta-hydroxybutyric acid was decreased in FA fibroblasts as compared to control cells. At the same time utilization of acetyl-L-carnitine and palmitoyl-D,L-carnitine was increased in FA fibroblasts. Utilization of L-glutamic acid, L-glutamine, L-ornithine, glycine/alanine and tryptamine was decreased in FA fibroblasts.     

Novel approach for evaluation of mitochondrial substrate utilization in fibroblasts from patients with Friedreich’s ataxia