Altered expression pattern of polycystin-2 in acute and chronic renal tubular diseases

• Verfasst von: Obermüller, Nicholas [VerfasserIn]
• Verfasst von: Cai, Yiqiang [VerfasserIn]
• Verfasst von: Kränzlin, Bettina [VerfasserIn]
• Verfasst von: Thomson, R. Brent [VerfasserIn]
• Verfasst von: Gretz, Norbert [VerfasserIn]
• Verfasst von: Kriz, Wilhelm [VerfasserIn]
• Verfasst von: Somlo, Stefan [VerfasserIn]
• Verfasst von: Witzgall, Ralph [VerfasserIn]
• Titel: Altered expression pattern of polycystin-2 in acute and chronic renal tubular diseases
• Verf.angabe: Nicholas Obermüller, Yiqiang Cai, Bettina Kränzlin, R. Brent Thomson, Norbert Gretz, Wilhelm Kriz, Stefan Somlo, and Ralph Witzgall
• E-Jahr: 2002
• Jahr: [July 2002]
• Umfang: 10 S.
• Illustrationen: Illustrationen
• Fussnoten: Gesehen am 24.06.2022
• Titel Quelle: Enthalten in: American Society of NephrologyJournal of the American Society of Nephrology
• Ort Quelle: Washington, DC : American Society of Nephrology, 1990
• Jahr Quelle: 2002
• Band/Heft Quelle: 13(2002), 7, Seite 1855-1864
• ISSN Quelle: 1533-3450
• DOI: doi:10.1097/01.ASN.0000018402.33620.C7
• Datenträger: Online-Ressource
• Sprache: eng
• K10plus-PPN: 1807802582

Abstract

ABSTRACT. Polycystin-2 represents one of so far two proteins found to be mutated in patients with autosomal-dominant polycystic kidney disease. Evidence obtained from experiments carried out in cell lines and with native kidney tissue strongly suggests that polycystin-2 is located in the endoplasmic reticulum. In the kidney, polycystin-2 is highly expressed in cells of the distal and connecting tubules, where it is located in the basal compartment. It is not known whether the expression of polycystin-2 in the kidney changes or whether it can be manipulated under certain instances. Therefore, the distribution of polycystin-2 under conditions leading to acute and chronic renal failure was analyzed. During ischemic acute renal failure, which affects primarily the S3 segment of the proximal tubule, a pronounced upregulation of polycystin-2 and a predominantly combined homogeneous and punctate cytoplasmic distribution in damaged cells was observed. After thallium-induced acute injury to thick ascending limb cells, polycystin-2 staining assumed a chicken wire-like pattern in damaged cells. In the (cy/+) rat, a model for autosomal-dominant polycystic kidney disease in which cysts originate predominantly from the proximal tubule, polycystin-2 immunoreactivity was lost in some distal tubules. In kidneys from (pcy/pcy) mice, a model for autosomal-recessive polycystic kidney disease in which cyst formation primarily affects distal tubules and collecting ducts, a minor portion of cyst-lining cells cease to express polycystin-2, whereas in the remaining cells, polycystin-2 is retained in their basal compartment. Data show that the expression and cellular distribution of polycystin-2 in different kinds of renal injuries depends on the type of damage and on the nephron-specific response to the injury. After ischemia, polycystin-2 may be upregulated by the injured cells to protect themselves. It is unlikely that polycystin-2 plays a role in cyst formation in the (cy/+) rat and in the (pcy/pcy) mouse.