title: Transcriptional adaptation to Clcn5 knockout in proximal tubules of mouse kidney.
abstract: 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.
authors: Department of Medicine, Johns Hopkins School of Medicine, Johns Hopkins University, Baltimore, Maryland, USA.
authors: Jerry
authors: J
authors: Wright
authors: Marcelo M
authors: MM
authors: Morales
authors: Jackson
authors: J
authors: Sousa-Menzes
authors: Debora
authors: D
authors: Ornellas
authors: Jennifer
authors: J
authors: Sipes
authors: Yan
authors: Y
authors: Cui
authors: Isabelle
authors: I
authors: Cui
authors: Phuson
authors: P
authors: Hulamm
authors: Valeriu
authors: V
authors: Cebotaru
authors: Liudmila
authors: L
authors: Cebotaru
authors: William B
authors: WB
authors: Guggino
authors: Sandra E
authors: SE
authors: Guggino
mesh_terms: D000222:Adaptation, Physiological / Q000235:genetics*; D000818:Animals; D018118:Chloride Channels / Q000172:deficiency* / Q000235:genetics* / Q000378:metabolism; D004195:Disease Models, Animal; D020869:Gene Expression Profiling; D005786:Gene Expression Regulation; D040181:Genetic Diseases, X-Linked; D007674:Kidney Diseases / Q000235:genetics* / Q000378:metabolism; D007687:Kidney Tubules, Proximal / Q000378:metabolism*; D050356:Lipid Metabolism / Q000235:genetics; D051379:Mice; D018345:Mice, Knockout; D009928:Organ Specificity; D020133:Reverse Transcriptase Polymerase Chain Reaction
chemical_list: C097503:CLC-5 chloride channel; D018118:Chloride Channels