caa a22 4500 397574665 CHVBK 20180308164852.0 cr unu---uuuuu 161202e199609 xx s 000 0 eng 10.1093/hmg/5.9.1299 doi (NATIONALLICENCE)oxford-10.1093/hmg/5.9.1299 Reduced Levels of Dystrophin Associated Proteins in the Brains of Mice Deficient for Dp71 [Elektronische Daten] [David S. Greenberg, Yechezkel Schatz, Zehava Levy, Paola Pizzo, David Yaffe, Uri Nudel] Duchenne muscular dystrophy (DMD) is a progressive degenerative lethal muscle disease. A significant proportion of DMD affected children suffer also from mental retardation. The rod shaped protein, dystrophin, which is absent from or defective in the muscle of DMD patients, binds to a number of membrane associated proteins (known collectively as dystrophin associated proteins [DAPs]). The levels of DAPs is greatly reduced in the muscle of DMD patients and mdx mice, which lack dystrophin. In addition to dystrophin isoforms, the DMD gene codes also for several smaller proteins. One of the small proteins, Dp71, is expressed in most or all non-muscle tissues and is the major DMD gene product in the brain. The function of the small DMD gene products is unknown. Here we show that mutant mice which do not express the smaller non-muscle products of the DMD gene have a reduced level of DAPs in their brain. This suggests that Dp71 is important for the formation and/or stabilization of a DAPs complex in brain. © 1996 Oxford University Press Greenberg David S. Department of Molecular Cell Biology, Weizmann Institute of Science, Rehovot, Israel aut Schatz Yechezkel Department of Molecular Cell Biology, Weizmann Institute of Science, Rehovot, Israel aut Levy Zehava Department of Molecular Cell Biology, Weizmann Institute of Science, Rehovot, Israel aut Pizzo Paola Department of Molecular Cell Biology, Weizmann Institute of Science, Rehovot, Israel aut Yaffe David Department of Molecular Cell Biology, Weizmann Institute of Science, Rehovot, Israel aut Nudel Uri Department of Molecular Cell Biology, Weizmann Institute of Science, Rehovot, Israel aut Human Molecular Genetics Oxford University Press 5/9(1996-09), 1299-1303 0964-6906 5:9<1299 1996 5 hmg https://doi.org/10.1093/hmg/5.9.1299 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1093/hmg/5.9.1299 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Greenberg David S. Department of Molecular Cell Biology, Weizmann Institute of Science, Rehovot, Israel aut NATIONALLICENCE 700 1- Schatz Yechezkel Department of Molecular Cell Biology, Weizmann Institute of Science, Rehovot, Israel aut NATIONALLICENCE 700 1- Levy Zehava Department of Molecular Cell Biology, Weizmann Institute of Science, Rehovot, Israel aut NATIONALLICENCE 700 1- Pizzo Paola Department of Molecular Cell Biology, Weizmann Institute of Science, Rehovot, Israel aut NATIONALLICENCE 700 1- Yaffe David Department of Molecular Cell Biology, Weizmann Institute of Science, Rehovot, Israel aut NATIONALLICENCE 700 1- Nudel Uri Department of Molecular Cell Biology, Weizmann Institute of Science, Rehovot, Israel aut NATIONALLICENCE 773 0- Human Molecular Genetics Oxford University Press 5/9(1996-09), 1299-1303 0964-6906 5:9<1299 1996 5 hmg Metadata rights reserved CC BY-NC-4.0 http://creativecommons.org/licenses/by-nc/4.0 nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-oxford