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Welcome everybody. My name is Edmund Kunji.
I work for the Medical Research Council
Mitochondrial Biology Unit of the University of Cambridge,
and today I'm going to talk about
Mitochondrial Transporters and how they're involved in their disease.
In my first talk,
I'm going to set the scene explaining what
mitochondrial carriers are and what they do in human physiology,
and then I'll go on and explain
the basic mechanism how we believe that they are actually working.
In my second talk,
I will explain the structural mechanism in much more detail,
and how these transporters are regulated.
But first, to set the scene,
I will explain what mitochondria actually are.
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Mitochondria are organelles in the human cell.
What you see here is a closeup inside a human cell,
which contains a large number of different organelles.
To the right-hand top corner,
what you find is the nucleus,
this is where the genetic information is stored.
Just below that it's the endoplasmic reticulum,
where proteins are synthesized with the information obtained from
the genetic material and where they are shipped to other parts of the cell.
In the left-hand upper corner,
you'll find the golgi apparatus,
this is the post office of the human cell where proteins are
transported to and sorted to go into
different directions in the cell. Then you find here
on the left-hand bottom,
you find a lysosome,
this is where proteins are degraded to be recycled for other purposes.
Then here in the front,
you find on the right-hand side,
right and bottom side, the close up of what we call the mitochondria.
Now the mitochondria are the powerhouses of the cell,
but this I will show you in a minute,
they do a lot more than that.
What you also find is that these mitochondria actually form large networks,
that are highly dynamic that are involved in fission,
the split up of these networks and fusion, diffusion of these networks,
and it's through all of these that they maintain
a high number of functional mitochondria in the cell.
Mitochondria are essential for many purposes as I will explain in a minute.
But first, let me tell you what the basic structure of a mitochondrion is.
So, this actually shows here,
