Article
LIMP-2/LGP85 deficiency causes ureteric pelvic junction obstruction, deafness and peripheral neuropathy in mice.
Human molecular genetics - 15 Mar 2003
Gamp Alexander-Christian, Tanaka Yoshitaka, Lüllmann-Rauch Renate, Wittke Dorothee, D'Hooge Rudi, De Deyn Peter P, Moser Tobias, Maier Hannes, Hartmann Dieter, Reiss Karina, Illert Anna-Lena, von Figura Kurt, Saftig Paul
Abstract excerpt
In previous overexpression studies we revealed a role for the lysosomal membrane protein LIMP-2/LGP85 in lysosomal biogenesis. LIMP-2-deficient mice show an increased postnatal mortality which is associated with a development of a uni- or bilateral hydronephrosis caused by an obstruction of the ureteropelvic junction. An accumulation of lysosomes in epithelial cells of the ureter adjacent to the ureteral lumen...
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