Altered expression pattern of polycystin-2 in acute and chronic renal tubular diseases
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...
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| Main Authors: | , , , , , , , |
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| Format: | Article (Journal) |
| Language: | English |
| Published: |
[July 2002]
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| In: |
Journal of the American Society of Nephrology
Year: 2002, Volume: 13, Issue: 7, Pages: 1855-1864 |
| ISSN: | 1533-3450 |
| DOI: | 10.1097/01.ASN.0000018402.33620.C7 |
| Online Access: | Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1097/01.ASN.0000018402.33620.C7 Verlag, lizenzpflichtig, Volltext: https://jasn.asnjournals.org/content/13/7/1855 
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| Author Notes: | Nicholas Obermüller, Yiqiang Cai, Bettina Kränzlin, R. Brent Thomson, Norbert Gretz, Wilhelm Kriz, Stefan Somlo, and Ralph Witzgall |
| Summary: | 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. |
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| Item Description: | Gesehen am 24.06.2022 |
| Physical Description: | Online Resource |
| ISSN: | 1533-3450 |
| DOI: | 10.1097/01.ASN.0000018402.33620.C7 |