On what basis can one conclude that the enzyme deficiency is associated with the electron transfer chain and not with the efficiency of proton translocation for ATP synthesis or translocation of adenine nucleotides across the inner mitochondrial membrane? Where is the enzyme defect located?

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On what basis can one conclude that the enzyme deficiency is associated with the electron
transfer chain and not with the efficiency of proton translocation for ATP synthesis or translocation of
adenine nucleotides across the inner mitochondrial membrane? Where is the enzyme defect located?

3. The following data compare respiratory rates of isolated mitochondria obtained from biopsy tissue
of patients with the same enzyme deficiency (uncertainties associated with measurement of respiratory
activities of patient biopsy tissue are comparable to control values):
Substrate
added
pyruvate + malate + ADP
glutamate + malate + ADP
succinate +rotenone + ADP
pyruvate + malate + ADP
Control
(normal)
92
+ 5
82 ± 7
104 + 11
1
12
10
96
+ dinitrophenol (DNP) 95 ± 5
92
(activity units are nanoatoms of oxygen consumed/min/mg protein)
Patients
2
29
24
98
96
3
19
19
103
93
4
13
26
99
95
Transcribed Image Text:3. The following data compare respiratory rates of isolated mitochondria obtained from biopsy tissue of patients with the same enzyme deficiency (uncertainties associated with measurement of respiratory activities of patient biopsy tissue are comparable to control values): Substrate added pyruvate + malate + ADP glutamate + malate + ADP succinate +rotenone + ADP pyruvate + malate + ADP Control (normal) 92 + 5 82 ± 7 104 + 11 1 12 10 96 + dinitrophenol (DNP) 95 ± 5 92 (activity units are nanoatoms of oxygen consumed/min/mg protein) Patients 2 29 24 98 96 3 19 19 103 93 4 13 26 99 95
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