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Fig. 1 | Human Genomics

Fig. 1

From: Hyperinsulinism associated with GLUD1 mutation: allosteric regulation and functional characterization of p.G446V glutamate dehydrogenase

Fig. 1

Mitochondrial metabolism in EBV-transformed lymphoblasts. The metabolic function of LCL lymphoblasts derived from control subject (GDH-wt) or from patient with mutant GDH (GDH-G446V) assessed using a 96-well plate Seahorse analyzer. Extracellular acidification rate (ECAR, a) and the oxygen consumption rate (OCR, b) were measured under the stimulation with 5 mM alanine and 5 mM glutamine, followed by the sequential addition of oligomycin (O), FCCP (F), and antimycin A/rotenone (A/R). ATP production (c) and maximal respiration (d) were calculated from OCR raw data. Values are shown as means ± SD, n = 10, *P < 0.05, **P < 0.01, ***P < 0.001 for wild-type GDH-wt versus GDH-G446V

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Human Genomics

ISSN: 1479-7364