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Paediatric Laboratory Medicine

Complex V

Clinical Significance

To determe the activity of respiratory chain complex V in isolated muscle and fibroblast mitochondria (ATPase hydrolysis)

Test Name

Complex V

Alternate Name/ Synonym

  • Oligomycin - sensitive ATPase

Gene Name

Caused by mutations in nuclear (eg TMEM70 (OMIM *612418), ATP53 (OMIM *606153) and mitochondrial genes (MTATP6, OMIM +516060 and MTATP8, OMIM +516070).


Mitochondrial Disease


Spectrophotometric Measurements at 340nm

Turn Around Time

  • Fibroblasts: 3 weeks - 3 months
  • Tissue Biopsies: Fresh - 5 business days


  • Systemic Mitochondrial Syndromes
  • Leigh's

Specimen Type

This assay is suitable only for testing on pure mitochondria (fibroblast or muscle)

Minimum Specimen Requirements

  • 20 dishes for mitochondrial isolation from fibroblasts
  • 250-300 mg for mitochondrial isolation from fresh muscle biopsy

Optimal Collection Time

  • For fibroblasts: anytime
  • Fresh biopsy: until 2:30pm, Monday-Friday


  • For fibroblasts: Room temperature
  • For fresh muscle biopsy: transport from Pathology in dish on ice

Special Requirements

For fresh biopsy: call to book (ext.205989) at least 24 hours in advance for full Mitochondrial Enzyme screening on isolated mitochondria. Pick up from Pathology.

Shipping and Contact Information

The Hospital for Sick Children
Rapid Response Laboratory
170 Elizabeth Street, Room 3642
Toronto, ON
M5G 2G3
Phone: 416-813-7200
Phone: 1-855-381-3212

Reference Range

  • Fibroblast mitochondria: 160.26-716.85 nmol/mg/min, CV/CS: 5.07-11.35
  • Muscle mitochondria: 466.61-2026.52 nmol/mg/min, CV/CS: ≥0.5