The structure, formation, and regulation of the respiratory chain is complex and the diagnosis of defects in oxidative phosphorylation (OXPHOS) is a major challenge. Traditional, spectrophotometric assays that measure the activity of individual respiratory complexes in tissue samples are limited by their sensitivity and the availability of sufficient biological material for testing. XF Bioenergetic Assays with patient fibroblast cultures overcome this limitation and have the advantages of being less affected by tissue specificity and the attenuation of defects detected spectrophotometrically in muscle or liver that disappear in cultured fibroblasts. Massimo Zeviani, MD, PhD, and Valeria Tiranti, PhD, use fibroblast cell lines derived from patients with OXPHOS deficiencies, and the Seahorse XF96 Analyzer to obtain a direct measure of cellular bioenergetics in a microplate.
IN THIS WEBINAR WE WILL DISCUSS
Seahorse XF96 assays on fibroblasts with genetically proven defects in OXPHOS-related genes.
How the Seahorse XF Analyzer can reveal mitochondrial dysfunction in fibroblasts with normal spectrophotometric activities, from patients with documented defects in muscle or OXPHOS-specific mutations.
The advantages of the scale-down in the amount of cultured cells needed for the diagnostic workout.
RECORDED
September 27, 2011
TYPE
On Demand
LANGUAGE
English
For Research Use Only. Not for use in diagnostic procedures.
PRESENTED BY
Massimo Zeviani, MD, PhD
The Foundation 'Carlo Besta' Institute of Neurology
Valeria Tiranti, PhD
The Foundation 'Carlo Besta' Institute of Neurology
Subscribe and learn more about Seahorse XF TechnologyStay informed
