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dc.contributor.authorFelbor, Ute
dc.contributor.authorKessler, Benedikt
dc.contributor.authorMothes, Walther
dc.contributor.authorGoebel, Hans H.
dc.contributor.authorPloegh, Hidde L.
dc.contributor.authorBronson, Roderick T.
dc.contributor.authorOlsen, Bjorn R.
dc.date.accessioned2019-10-03T17:42:27Z
dc.date.issued2002
dc.identifier.citationFelbor, U., B. Kessler, W. Mothes, H. H. Goebel, H. L. Ploegh, R. T. Bronson, and B. R. Olsen. 2002. “Neuronal Loss and Brain Atrophy in Mice Lacking Cathepsins B and L.” Proceedings of the National Academy of Sciences 99 (12): 7883–88. https://doi.org/10.1073/pnas.112632299.
dc.identifier.issn0027-8424
dc.identifier.issn0744-2831
dc.identifier.issn1091-6490
dc.identifier.urihttp://nrs.harvard.edu/urn-3:HUL.InstRepos:41467502*
dc.description.abstractCathepsins B and L are widely expressed cysteine proteases implicated in both intracellular proteolysis and extracellular matrix remodeling. However, specific roles remain to be validated in vivo. Here we show that combined deficiency of cathepsins B and L in mice is lethal during the second to fourth week of life. Cathepsin beta(-/-)/L-/- mice reveal a degree of brain atrophy not previously seen in mice. This is because of massive apoptosis of select neurons in the cerebral cortex and the cerebellar Purkinje and granule cell layers. Neurodegeneration is accompanied by pronounced reactive astrocytosis and is preceded by an accumulation of ultrastructurally and biochemically unique lysosomal bodies in large cortical neurons and by axonal enlargements. Our data demonstrate a pivotal role for cathepsins B and L in maintenance of the central nervous system.
dc.language.isoen_US
dc.publisherNational Academy of Sciences
dash.licenseLAA
dc.titleNeuronal loss and brain atrophy in mice lacking cathepsins B and L
dc.typeJournal Article
dc.description.versionVersion of Record
dc.relation.journalProceedings of the National Academy of Sciences of the United States of America
dash.depositing.authorOlsen, Bjorn Reino::aed6a83ff845c000160456935386d060::600
dc.date.available2019-10-03T17:42:27Z
dash.workflow.comments1Science Serial ID 89433
dc.identifier.doi10.1073/pnas.112632299
dash.source.volume99;12
dash.source.page7883


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