The disease is characterized by the development of a plurality of fluid-filled cysts in both kidneys. This leads to kidney failure, usually between the ages of 50 to 60 years.
The researchers suggested that polycystic occurs due to dysfunction of the cilia of ciliated epithelium, which are not able to detect and display the accumulated flow of urine. An uncontrolled formation of cysts, eventually destroy the kidney.
Cilia are finger-like projections of the epithelial cells, which until recently was considered rudimentary last residue. To date, they have been recognized as the main sensor controls and regulates everything that passes through the cell.
It is known that ciliary dysfunction leads to a wide variety of eye diseases, hearing aids, and other heart. Bodies. Such a statement could be a representation of how best to treat and prevent these diseases.
"What is the structure of cilia? If you know it, you can figure out what went wrong and caused the diseases, including polycystic kidney disease, "says nephrologist Joshua Lipschutz - MD, author of articles.
Proteins necessary for ciliogenesis (formation of cilia), produced in the endoplasmic reticulum. They come into the Golgi apparatus - is formed by the membrane and the vacuole system of small bubbles through which the synthesized on membrane lipids and proteins. Even before arriving in the Golgi apparatus, they must be "sealed". Renal ciliogenesis occurs only when "packed proteins" are delivered to the protein complex. He should be in place and be prepared to obtain such proteins. It plays the role of the presence of the tubes, which are a kind of conductor. Without it delivered "packages" are accumulated, and because of this, the formation of cysts may occur.
Lipschutz and his co-Sec-Hyun Kim (PhD) guide study mice kidney, t. K. They are more similar to human others. When the causes underlying disorders ciliogenesis will be more fully understood, then remedial measures can be developed. "We are investigating this to help our patients, and further clarification is an important step in the development of treatment options," - said Dr. Lipschutz.
Based on materials sciencedaily.com