Mice deficient for the lysosomal proteinase cathepsin D exhibit progressive atrophy of the intestinal mucosa and profound destruction of lymphoid cells.

AUTOR(ES)

Saftig, P

RESUMO

Mice deficient for the major lysosomal aspartic proteinase cathepsin D, generated by gene targeting, develop normally during the first 2 weeks, stop thriving in the third week and die in a state of anorexia at day 26 +/- 1. An atrophy of the ileal mucosa first observed in the third week progresses towards widespread intestinal necroses accompanied by thromboemboli. Thymus and spleen undergo massive destruction with fulminant loss of T and B cells. Lysosomal bulk proteolysis is maintained. These results suggest, that vital functions of cathepsin D are exerted by limited proteolysis of proteins regulating cell growth and/or tissue homeostasis, while its contribution to bulk proteolysis in lysosomes appears to be non-critical.

Documentos Relacionados

• Dilated cardiomyopathy in mice deficient for the lysosomal cysteine peptidase cathepsin L
• A mutation in the ovine cathepsin D gene causes a congenital lysosomal storage disease with profound neurodegeneration
• Isolation of a cDNA clone for the human lysosomal proteinase cathepsin B.
• Ndrg1-Deficient Mice Exhibit a Progressive Demyelinating Disorder of Peripheral Nerves
• The effect of progressive immunization on polyribosomal size in lymphoid cells.