Fig. 3
From: Personalized medicine approach confirms a milder case of ABAT deficiency
Mitochondrial bioenergetics are compromised in ABAT deficiency patients. a. Mitochondrial membrane potential is reduced in fibroblasts from ABAT deficiency patients Subject 1 (S1), Subject2 (S2), and Subject3 (S3). b. Microscale oxygraphy demonstrated that ATP production, c. Basal respiration, d. Maximum respiration, and e. Reserve capacity were all relatively similarly compromised in ABAT deficiency patients, both mild (S1) and severe (S2, S3)