RGD Reference Report - Altered expression pattern of polycystin-2 in acute and chronic renal tubular diseases. - Rat Genome Database

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

Authors: Obermuller, N  Cai, Y  Kranzlin, B  Thomson, RB  Gretz, N  Kriz, W  Somlo, S  Witzgall, R 
Citation: Obermuller N, etal., J Am Soc Nephrol. 2002 Jul;13(7):1855-64.
RGD ID: 7175293
Pubmed: PMID:12089381   (View Abstract at PubMed)

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.

RGD Manual Disease Annotations    Click to see Annotation Detail View
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
acute kidney failure  ISOPkd2 (Rattus norvegicus)7175293; 7175293protein:altered localizationRGD 
acute kidney failure  IEP 7175293protein:altered localizationRGD 

Objects Annotated

Genes (Rattus norvegicus)
Pkd2  (polycystin 2, transient receptor potential cation channel)

Genes (Mus musculus)
Pkd2  (polycystin 2, transient receptor potential cation channel)

Genes (Homo sapiens)
PKD2  (polycystin 2, transient receptor potential cation channel)


Additional Information