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0:00
Hello and welcome.
My name is Paul Jennings.
I'm an Assistant Professor
at the Division of Physiology
in the Medical University of Innsbruck.
And today, I'd like to present
the Molecular Basis
of Genetic Renal Diseases.
0:15
The kidney is responsible
for whole body homeostasis.
It receives about 20%
of the cardiac output,
meaning that nearly 1,500 liters per day
are profused through the kidney.
And of this, 180 liters are filtered
through the kidney,
producing about
1 to 2 liters of urine per day.
Through this system,
water solutes including glucose, ions,
amino acids, and proteins,
and vitamins are reabsorbed.
Excess nutrients and waste products
and xenobiotics are eliminated.
In addition, the kidney
is extremely important
in the regulation
of the acid base balance
and the regulation of vitamin D
and the erythropoietin.
0:58
The functional unit
of the kidney is the nephron,
and here, I've drawn a cartoon of this
where the blood
leaves the endothelial cells
into the glomerulus,
and then from the glomerulus
into the proximal tubule,
into the thin descending
limb, the thick ascending limb,
the distal tubule,
and then the collecting duct.
Each region depicted here
has a different set of transporters
and proteins
which means that each region handles
glomerular filtrates differently.
1:28
The kidney unlike, for example,
the liver has a limited
regenerative capacity.
As I said, we started off
with approximately
1.5 million nephrons per kidney.
However, nephrons lost throughout life
due to processes
such as oxidative damage
and there is no de novo nephrogenesis
after birth.
There is also little
substantial evidence
for the existence
of adult renal progenitor cells.
Thus repair seems to be intrinsic
through the nephron itself and limited.
The functional capacity of the kidney
is several times more than required
which poses a problem in that
whole body homeostasis
is not compromised
until the majority of nephrons are lost,
and by that stage, it's usually
too late to do anything about it.
