Deficiency of the tensin2 gene in the ICGN mouse: An animal model for congenital nephrotic syndrome

A. Ri Cho, Kozue Uchio-Yamada, Takeshi Torigai, Tomomi Miyamoto, Ichiro Miyoshi, Junichiro Matsuda, Tsutomu Kurosawa, Yasuhiro Kon, Atsushi Asano, Nobuya Sasaki, Takashi Agui

Research output: Contribution to journalArticlepeer-review

45 Citations (Scopus)


The ICGN mouse is a model for nephrotic syndrome (NS) which presents with proteinuria, hyperlipidemia, and edema. In this study we attempted to identify the gene(s) responsible for NS. By analyzing albuminuria in 160 (ICGN × MSM)F1 × ICGN backcross progenies, we found that NS in the ICGN mouse is caused by more than one gene. We then performed a quantitative trait locus (QTL) analysis and detected a QTL with a very high LOD score peak in the telomeric region of Chr 15. By analyzing the nucleotide sequence of 22 genes located close to the QTL, we found that the tensin2 gene of the ICGN mouse possessed an 8-nucleotide deletion mutation in exon 18, leading to a frameshift and giving rise to a terminal codon at a premature position. Analyses of in situ hybridization and immunohistochemistry revealed that tensin2 was expressed in podocytes and tubular epithelial cells in normal mice but not in the ICGN mouse. These data raise the possibility that a mutation of the tensin2 gene is responsible for NS of the ICGN mouse and tensin2 is a prerequisite for the normal kidney function.

Original languageEnglish
Pages (from-to)407-416
Number of pages10
JournalMammalian Genome
Issue number5
Publication statusPublished - 2006 May


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