Transcriptional adaptation to Clcn5 knockout in proximal tubules of mouse kidney

Jerry Wright, Marcelo M. Morales, Jackson Sousa-Menzes, Debora Ornellas, Jennifer Sipes, Yan Cui, Isabelle Cui, Phuson Hulamm, Valeriu Cebotaru, Liudmila Cebotaru, William B. Guggino, Sandra E. Guggino

Research output: Contribution to journalArticlepeer-review

22 Scopus citations


Dent disease has multiple defects attributed to proximal tubule malfunction including low-molecular-weight proteinuria, aminoaciduria, phosphaturia, and glycosuria. To understand the changes in kidney function of the Clc5 chloride/proton exchanger gene knockout mouse model of Dent disease, we examined gene expression profiles from proximal S1 and S2 tubules of mouse kidneys. We found many changes in gene expression not known previously to be altered in this disease. Genes involved in lipid metabolism, organ development, and organismal physiological processes had the greatest number of significantly changed transcripts. In addition, genes of catalytic activity and transporter activity also had a great number of changed transcripts. Overall, 720 genes are expressed differentially in the proximal tubules of the Dent Clcn5 knockout mouse model compared with those of control wild-type mice. The fingerprint of these gene changes may help us to understand the phenotype of Dent disease.

Original languageEnglish (US)
Pages (from-to)341-354
Number of pages14
JournalPhysiological Genomics
Issue number3
StatePublished - May 2008


  • Cholesterol
  • Dent disease
  • Endocytosis
  • Gene array

ASJC Scopus subject areas

  • Physiology
  • Genetics


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