Molecular basis of genetic renal diseases 1

Published on December 28, 2016   27 min

Other Talks in the Series: The Kidney in Health and Disease

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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.
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.
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.
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.