Abnormal regulation of the Ke 6 gene, a new 17β-hydroxysteroid dehydrogenase in the cpk mouse kidney

Sylvia Ramirez, Ioulia Fomitcheva, Nazneen Aziz

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

The function encoded by the Ke 6 gene has been recently determined to be 17β-hydroxysteroid dehydrogenase. Previously, the abnormal expression of the Ke 6 gene has been intimately associated with development of recessive polycystic kidney disease. The Ke 6 gene is normally expressed at very high levels in the kidney and liver and is severely down regulated in all recessive murine models of polycystic kidney disease that have been examined to date. Here, we report a detailed examination of the promoter region of the Ke 6 gene in normal mouse kidney cells (CTA) and in cells derived from mouse kidneys homozygous for the cpk (congenital polycystic kidney) mutation, using transfection analysis and DNA-protein gel shift assays. The minimal promoter region, P1 (+1 to -96), and a putative enhancer site, P3 (-165 to -256), within the Ke 6 gene 5' flanking sequence have been identified. We have also identified another region, P2 (-97 to -165), that may be responsible for the lower promoter activity of the Ke 6 gene in cpk cells, Furthermore, absence of binding of a 38 kDa nuclear protein to a 16 bp sequence element (PIA) within the minimal promoter of the Ke 6 gene suggests that the P1A element could be responsible for the overall reduction in promoter function in cpk cells.

Original languageEnglish (US)
Pages (from-to)9-22
Number of pages14
JournalMolecular and Cellular Endocrinology
Volume143
Issue number1-2
DOIs
StatePublished - Aug 25 1998

Fingerprint

Polycystic Kidney Diseases
Genes
Kidney
Genetic Promoter Regions
5' Flanking Region
Nuclear Proteins
3(17)-hydroxysteroid dehydrogenase
Liver
Transfection
Assays
Gels
Mutation
DNA

Keywords

  • Ke 6 gene
  • Promoter activity
  • cpk cells

ASJC Scopus subject areas

  • Biochemistry
  • Molecular Biology
  • Endocrinology

Cite this

Abnormal regulation of the Ke 6 gene, a new 17β-hydroxysteroid dehydrogenase in the cpk mouse kidney. / Ramirez, Sylvia; Fomitcheva, Ioulia; Aziz, Nazneen.

In: Molecular and Cellular Endocrinology, Vol. 143, No. 1-2, 25.08.1998, p. 9-22.

Research output: Contribution to journalArticle

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