Nephron. Experimental nephrology · 2013
Tenc1-deficient mice develop glomerular disease in a strain-specific manner.
СтатьяПоддержка исследования: вне правительства СШАНазвание и аннотация: Перевод в очереди
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Uchio-Yamada Kozue, Sawada Kyoko, Tamura Kotaro, Katayama Sumie, Monobe Youko, Yamamoto Yoshie, Ogura Atsuo, Manabe Noboru
Аннотация
BACKGROUND/AIMS: Tenc1 (also known as tensin2) is an integrin-associated focal adhesion molecule that is broadly expressed in mouse tissues including the liver, muscle, heart and kidney. A mouse strain carrying mutated Tenc1, the ICR-derived glomerulonephritis (ICGN) strain, develops severe nephrotic syndrome.
METHODS: To elucidate the function of Tenc1 in the kidney, Tenc1(ICGN) was introduced into 2 genetic backgrounds, i.e. DBA/2J (D2) and C57BL/6J (B6), strains that are respectively susceptible and resistant to chronic kidney disease.
RESULTS: Biochemical and histological analysis revealed that homozygous Tenc1(ICGN) mice develop nephrotic syndrome on the D2 background (D2GN) but not on the B6 background (B6GN). Initially, abnormal assembly and maturation of glomerular basement membrane (GBM) were observed, and subsequently effacement of podocyte foot processes was noted in the kidneys of D2GN but not B6GN mice. These defects are likely to be involved in the integrin signaling pathway.
CONCLUSION: This study suggests that Tenc1 contributes to the maintenance of GBM structures and that the genetic background influences the severity of nephrotic syndrome.
METHODS: To elucidate the function of Tenc1 in the kidney, Tenc1(ICGN) was introduced into 2 genetic backgrounds, i.e. DBA/2J (D2) and C57BL/6J (B6), strains that are respectively susceptible and resistant to chronic kidney disease.
RESULTS: Biochemical and histological analysis revealed that homozygous Tenc1(ICGN) mice develop nephrotic syndrome on the D2 background (D2GN) but not on the B6 background (B6GN). Initially, abnormal assembly and maturation of glomerular basement membrane (GBM) were observed, and subsequently effacement of podocyte foot processes was noted in the kidneys of D2GN but not B6GN mice. These defects are likely to be involved in the integrin signaling pathway.
CONCLUSION: This study suggests that Tenc1 contributes to the maintenance of GBM structures and that the genetic background influences the severity of nephrotic syndrome.
Предметные рубрики MeSH
Adaptor Proteins, Signal TransducingAnimalsBlotting, WesternCollagen Type IVCytoskeletal ProteinsGlomerular Basement MembraneGlomerulonephritisIntegrin alpha3beta1Kidney GlomerulusLamininMembrane ProteinsMiceMice, Inbred C57BLMice, Inbred DBAMice, Inbred ICRMice, KnockoutMicroscopy, Electron, TransmissionMicroscopy, FluorescenceNephrotic SyndromePhosphoprotein PhosphatasesPodocytesProteinuriaSpecies SpecificityTensins