82657 x 6 (six assays are performed)
88233 x 1
88240 x 1
Monday - Friday, 0800 - 1700.
2 - 3 months (depending on growth rate of cultured cells).
Two T-25 flasks of confluent fibroblasts, with plug-seal caps, completely filled with media. Fibroblasts must be mycoplasma-free.
Send flasks in a Styrofoam box at room temperature. Please include passage number (if known).
A requisition form for mitochondrial enzyme assay must be enclosed. Please complete both
pages of the requisition form for mitochondrial enzyme assays.
Interpretive Reference Range (5th and 95th percentiles):
Fibroblasts (in nmol/min per mg protein):
Complex 1: 48.9–120.1
Complex 2: 144.4-349.2
Complex 3: 7.92-30.38
Complex 2+3: 102.1 -210.6
Complex 4: 2.13 -6.84
Citrate synthase: 245.4 -540.4
(Ratios of complex activities over citrate synthase and over complex 2 are provided. Subject to interpretation.)
Diagnosis of mitochondrial respiratory chain diseases
Spectrophotometric enzyme assays