TABLE 1.

[¹³C] labeled metabolite concentrations determined by NMR spectroscopy in IBAT mitochondrial incubates

Metabolite	Nucleotide presence (+) or absence (−)				Nucleotide effect (p‐value)
	ADP−, GDP−	ADP−, GDP+	ADP+, GDP−	ADP+, GDP+	ADP	GDP
Oxaloacetate (µM)	0 ± 0	0 ± 0	0 ± 0	0 ± 0	n/a	n/a
Malate (µM)	90 ± 3	193 ± 24aa	100 ± 5	128 ± 10b	0.055*	<0.001*
Aspartate (µM)	0 ± 0	0 ± 0	0 ± 0	0 ± 0	n/a	n/a
Citrate (µM)	277 ± 15	267 ± 13	263 ± 20	214 ± 13	0.051	0.082
α‐ketoglutarate (µM)	162 ± 21	59 ± 7a	221 ± 23	203 ± 19bb	< 0.001*	0.007*

Mitochondria were energized by 5 mM pyruvate +1 mM [U‐¹³C] malate and incubated for 20 min under the same conditions used in the studies of Figure 2. Data were analyzed by 2‐factor (ADP × GDP) ANOVA. n = 4 for all values. All metabolites were determined in the same 4 individual mitochondrial preparations.

^a p< 0.01, ^aa p < 0.001 versus ADP‐, GDP‐, ^b p< 0.01, ^bb p < 0.001 versus ADP‐, GDP+.

^*Interaction was significant, so interpretation is limited. Data represent mean ± SE.