RGD Reference Report - Increased activity of activator protein-1 transcription factor components ATF2, c-Jun, and c-Fos in human and mouse autosomal dominant polycystic kidney disease. - Rat Genome Database

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Increased activity of activator protein-1 transcription factor components ATF2, c-Jun, and c-Fos in human and mouse autosomal dominant polycystic kidney disease.

Authors: Le, NH  Van der Wal, A  Van der Bent, P  Lantinga-van Leeuwen, IS  Breuning, MH  Van Dam, H  De Heer, E  Peters, DJ 
Citation: Le NH, etal., J Am Soc Nephrol. 2005 Sep;16(9):2724-31. Epub 2005 Jul 27.
RGD ID: 7242198
Pubmed: PMID:16049073   (View Abstract at PubMed)
DOI: DOI:10.1681/ASN.2004110913   (Journal Full-text)

Autosomal dominant polycystic kidney disease is a common inherited disorder that predominantly manifests with the formation of fluid-filled cysts in both kidneys. The disease can be accounted for by a mutation in either the PKD1 or the PKD2 gene. It was demonstrated previously that aberrant expression of the PKD1 gene product, polycystin-1, results in modification of activator protein-1 (AP-1) transcription factor activity in cultured renal epithelial cells. Here, it is reported that activity of the AP-1 components c-Jun, ATF2, and c-Fos is altered in renal cystic tissue of patients with autosomal dominant polycystic kidney disease and of hypomorphic Pkd1 mice with polycystic kidney disease. Data were obtained using immunohistochemical and Western blot analysis. Significant upregulation of Thr71- and Thr69/71-phosphorylated ATF2 and Ser73-phosphorylated c-Jun and increased c-Fos were detected in small cysts and (dilated) ducts and tubules surrounded by fibrotic interstitium. The data indicate that various AP-1 components are constitutively activated in polycystic kidney disease and suggest that aberrant AP-1 activity is relevant for cyst formation.

RGD Manual Disease Annotations    Click to see Annotation Detail View
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
autosomal dominant polycystic kidney disease  IEP 7242198protein: increased expression: kidneyRGD 
autosomal dominant polycystic kidney disease  ISOFOS (Homo sapiens)7242198; 7242198protein: increased expression: kidneyRGD 

Objects Annotated

Genes (Rattus norvegicus)
Fos  (Fos proto-oncogene, AP-1 transcription factor subunit)

Genes (Mus musculus)
Fos  (FBJ osteosarcoma oncogene)

Genes (Homo sapiens)
FOS  (Fos proto-oncogene, AP-1 transcription factor subunit)


Additional Information