Mitochondrial oxidative phosphorylation in autosomal dominant optic atrophy

BMC Biochemistry

Table 2 Polarographic analysis of ADOA patient and control mitochondria

	Mean respiration rates and ADP/O with substrates indicated^a nmol O/min/mg mitochondrial protein
	Malate + Pyruvate			Glutamate + Malate			Succinate
	III	IV	UC	III	IV	UC	III	IV	UC
^bOPA1+ (n = 6)
P2	211	43	258	235	52	270	313	65	349
P3	349	58	442	398	50	474	537	97	653
P4	290	41	397	310	36	392	393	76	468
P6	173	36	280	170	31	276	276	54	372
P7	281	48	362	310	41	348	356	73	403
P8	250	44	322	280	35	338	310	57	391
Mean ± SD	259 ± 62	45 ± 8	344 ± 70	284 ± 77	41 ± 9	350 ± 76	364 ± 94	70 ± 16	439 ± 112
^bOPA1- (n = 10)
Mean ± SD	230 ± 64	38 ± 13	309 ± 99	270 ± 88	39 ± 18	312 ± 145	301 ± 83	58 ± 22	356 ± 124
Control (n = 10)
Mean ± SD	300 ± 37	49 ± 12	370 ± 73	312 ± 38	45 ± 12	328 ± 98	376 ± 66	66 ± 18	421 ± 63

^aAbbreviations used for respiratory rate data are III, state III rate; IV, state IV rate; and UC, uncoupled rate.
^bAbbreviations used for patient genetics are OPA1+, OPA1-positive patients and OPA1-,OPA1-negative patients

ISSN: 1471-2091