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0:00
Hello, my name is Monika Winter,
and I am an assistant
professor in
biochemistry at the
Faculty of Health
and Life Sciences at Northhumbria
University in the UK.
Today, I'm going to
be talking to you
about the aetiology and
the molecular genetic basis of
oxidative phosphorylation
deficiency.
0:22
Mitochondria play
an essential role in
cellular metabolism.
But what is cellular metabolism?
We can define it as
molecular
transformations that are
occurring in living cells,
which involves
coordinated series of
chemical reactions in order
to produce or consume energy.
But basically, in other words,
metabolism represents
this balance
between the catabolic and
anabolic pathways in a cell.
Catabolic pathways
are essentially
the pathways in which
we intake food.
This food is then broken
down in our bodies.
As a result, it
results in a number of
useful constituents,
one of which is energy,
and another one are
cellular building blocks.
The energy and the building
blocks can be used by
the anabolic pathways to
make various macromolecules
such as DNA,
RNA, lipid, and those
macromolecules are,
of course, very important
building blocks
of our organelles,
cells, tissues, and
organs. Metabolism,
as I said, really represents
a balance between
these two pathways.
1:40
Metabolism is very complicated.
It involves a number of
different pathways that
are highly interconnected.
On this illustration,
you can see a circuitry
of chemical reactions which
occur inside our cells.
These link all the possible
metabolisms together,
not only the ones
that break down food,
but also the ones that are
building blocks for our life.
All the food sources
break down in a way
that allows the production
of simple carbon sources.
These carbon sources
flow through what
is known as the TCA
or the Krebs cycle,
which produces
reducing equivalents.
These reducing
equivalents, which
are captured from the TCA cycle,
but also other pathways,
are the ones that feed into
the mitochondrial oxidative
phosphorylation system.
This system is a chain of
macromolecular complexes that is
embedded in the inner
mitochondrial membrane.
These complexes
constantly shuttle
electrons in exchange
for transporting
proton to generate an
electrochemical gradient
that is used to produce
adenosine triphosphate or ATP.
