0:00
My name is Paul Gissen,
and I'm a researcher at the UCL
Laboratory for
Molecular Cell Biology
and Institute of Child Health.
And I'm also a consultant of
metabolic medicine in Great
Ormond Street Hospital for Children.
Today I'm going to talk to you
about traffic problems, inherited
disease, and intracellular
trafficking defect.
0:24
In this talk, we will
discuss concepts and pathways
in intracellular
protein trafficking.
We will also mention different
disorders that are caused
by specific trafficking
abnormalities.
I will also focus on ARC syndrome
as an example of a trafficking
disorder, as this is the
interest of my laboratory.
0:51
Intracellular protein trafficking
is broadly divided into two steps,
and they are directly
opposite to each other.
One of them is protein secretion,
in which polypeptides which
are synthesized in the ribosomes
are then folded and oligomerized
in the ER and then packaged
into transport vesicles
in the endoplasmic reticulum
and travel to Golgi complex.
In the Golgi, it's organ where post
translational modification occurs,
and then the proteins are
sorted into various routes
and then directed in various
vesicles towards the membrane
or other organelles of the cell.
The endocytosis is where the
proteins are internalized
from the plasma
membrane, and then they
can be cycled back into the
membrane or maybe trafficked
with degradation to lysosomes or
transcytosed from the basolateral
to the apical membrane of the cell.
