My name is Eamonn Maher, I'm Professor of
Medical Genetics and Genomic Medicine and
Honorary Consultant in Clinical Genetics
in the Department of Medical Genetics at
the University of Cambridge and
Cambridge University Hospitals.
I'm going to be speaking to you on
mitochondria in health and disease.
Mitochondria are essential
cellular organelles that,
with the exception of red blood cells,
occur nearly all eukaryotic cells.
The number of mitochondria per cell
varies from hundreds to thousands,
and is higher in the more
metabolically active tissues.
The best-known role for mitochondria
is to generate energy for the cell,
in particular the generation of
ATP by oxidative phosphorylation.
However, mitochondria may also have
roles in other cellular processes such
as apoptosis, heat production, amino
acid metabolism and lipid metabolism.
In this slide, we see
the organisation of the mitochondria,
they're organised into four compartments.
There's a smooth outer membrane that
is freely permeable to ions and small
molecules and an inner folded membrane
that encloses the matrix space, the inner
membrane folds are known as cristae and
they're impermeable to small molecules.
The compartment between the inner and
outer membranes is known
as the intermembrane space.
The innermost part (the matrix)
contains the mitochondrial DNA and
proteins involved in biochemical pathways,
such as the tricarboxylic acid or
Krebs cycle pathway.
Within the matrix, the multimeric
complexes of the respiratory
chain convert energy derived
from nutrients into ATP,
in a process known as
More than 1,500 proteins are required for
mitochondria to function.