Table 1 Mitochondrial Oxygen Consumption Rates (OCR) in intact MCAD-deficient patient fibroblasts.

From: Loss of the Mitochondrial Fatty Acid β-Oxidation Protein Medium-Chain Acyl-Coenzyme A Dehydrogenase Disrupts Oxidative Phosphorylation Protein Complex Stability and Function

 

Mitochondrial Oxygen Consumption Rates pmol/min/20,000 cells (% of Control Values)

OCR-Basal

OCR-O

OCR-F

OCR-R

FCCP-stimulated

Control

51 ± 12

19 ± 5

89 ± 24

15 ± 6

74 ± 19

Patient 1 (P1)

28 ± 5* (55%)

11 ± 2* (57%)

42 ± 9* (47%)

9 ± 3* (63%)

32 ± 8* (44%)

Patient 2 (P2)

40 ± 8* (78%)

14 ± 3* (73%)

53 ± 11* (60%)

12 ± 2 (79%)

42 ± 9* (56%)

  1. OCR-O = OCR with oligomycin; OCR-F = OCR with FCCP; OCR-R = OCR with rotenone.
  2. FCCP-stimulated = OCR-F minus OCR-R.
  3. Each condition was performed in at least 14 wells on the same plate.
  4. *p < 0.01.
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