Acute pathophysiological effects of muscle-expressed Dp71 transgene on normal and dystrophic mouse muscle
Wieneke S, Heimann P, Leibovitz S, Nudel U, Jockusch H (2003)
Journal of Applied Physiology 95(5): 1861-1866.
Zeitschriftenaufsatz
| Veröffentlicht | Englisch
Download
Es wurden keine Dateien hochgeladen. Nur Publikationsnachweis!
Autor*in
Wieneke, S;
Heimann, PeterUniBi;
Leibovitz, S;
Nudel, U;
Jockusch, HaraldUniBi
Einrichtung
Abstract / Bemerkung
The products of the dystrophin gene range from the 427-kDa full-length dystrophin to the 70.8-kDa Dp71. Dp427 is expressed in skeletal muscle, where it links the actin cytoskeleton with the extracellular matrix via a complex of dystrophin-associated proteins (DAPs). Dystrophin deficiency disrupts the DAP complex and causes muscular dystrophy in humans and the mdx mouse. Dp71, the major nonmuscle product, consists of the COOH-terminal part of dystrophin, including the binding site for the DAP complex but lacks binding sites for microfilaments. Dp71 transgene (Dp71tg) expressed in mdx muscle restores the DAP complex but does not prevent muscle degeneration. In wild-type (WT) mouse muscle, Dp71tg causes a mild muscular dystrophy. In this study, we tested, using isolated extensor digitorum longus muscles, whether Dp71tg exerts acute influences on force generation and sarcolemmal stress resistance. In WT muscles, there was no effect on isometric twitch and tetanic force generation, but with a cytomegalovirus promotor-driven transgene, contraction with stretch led to sarcolemmal ruptures and irreversible loss of tension. In MDX muscle, Dp71tg reduced twitch and tetanic tension but did not aggravate sarcolemmal fragility. The adverse effects of Dp71 in muscle are probably due to its competition with dystrophin and utrophin ( in MDX muscle) for binding to the DAP complex.
Stichworte
dystrophin;
mechanophysiology;
eccentric contractions;
sarcolemmal ruptures;
mdx mouse
Erscheinungsjahr
2003
Zeitschriftentitel
Journal of Applied Physiology
Band
95
Ausgabe
5
Seite(n)
1861-1866
ISSN
0
Page URI
https://pub.uni-bielefeld.de/record/1609927
Zitieren
Wieneke S, Heimann P, Leibovitz S, Nudel U, Jockusch H. Acute pathophysiological effects of muscle-expressed Dp71 transgene on normal and dystrophic mouse muscle. Journal of Applied Physiology. 2003;95(5):1861-1866.
Wieneke, S., Heimann, P., Leibovitz, S., Nudel, U., & Jockusch, H. (2003). Acute pathophysiological effects of muscle-expressed Dp71 transgene on normal and dystrophic mouse muscle. Journal of Applied Physiology, 95(5), 1861-1866. https://doi.org/10.1152/japplphysiol.00326.2003
Wieneke, S, Heimann, Peter, Leibovitz, S, Nudel, U, and Jockusch, Harald. 2003. “Acute pathophysiological effects of muscle-expressed Dp71 transgene on normal and dystrophic mouse muscle”. Journal of Applied Physiology 95 (5): 1861-1866.
Wieneke, S., Heimann, P., Leibovitz, S., Nudel, U., and Jockusch, H. (2003). Acute pathophysiological effects of muscle-expressed Dp71 transgene on normal and dystrophic mouse muscle. Journal of Applied Physiology 95, 1861-1866.
Wieneke, S., et al., 2003. Acute pathophysiological effects of muscle-expressed Dp71 transgene on normal and dystrophic mouse muscle. Journal of Applied Physiology, 95(5), p 1861-1866.
S. Wieneke, et al., “Acute pathophysiological effects of muscle-expressed Dp71 transgene on normal and dystrophic mouse muscle”, Journal of Applied Physiology, vol. 95, 2003, pp. 1861-1866.
Wieneke, S., Heimann, P., Leibovitz, S., Nudel, U., Jockusch, H.: Acute pathophysiological effects of muscle-expressed Dp71 transgene on normal and dystrophic mouse muscle. Journal of Applied Physiology. 95, 1861-1866 (2003).
Wieneke, S, Heimann, Peter, Leibovitz, S, Nudel, U, and Jockusch, Harald. “Acute pathophysiological effects of muscle-expressed Dp71 transgene on normal and dystrophic mouse muscle”. Journal of Applied Physiology 95.5 (2003): 1861-1866.
Daten bereitgestellt von European Bioinformatics Institute (EBI)
UNIPROT
23 Einträge gefunden, die diesen Artikel zitieren von denen 10 angezeigt werden
2 Zitationen in Europe PMC
Daten bereitgestellt von Europe PubMed Central.
Upregulation of brain utrophin does not rescue behavioral alterations in dystrophin-deficient mice.
Perronnet C, Chagneau C, Le Blanc P, Samson-Desvignes N, Mornet D, Laroche S, De La Porte S, Vaillend C., Hum Mol Genet 21(10), 2012
PMID: 22343141
Perronnet C, Chagneau C, Le Blanc P, Samson-Desvignes N, Mornet D, Laroche S, De La Porte S, Vaillend C., Hum Mol Genet 21(10), 2012
PMID: 22343141
Impact of sarcoglycan complex on mechanical signal transduction in murine skeletal muscle.
Barton ER., Am J Physiol Cell Physiol 290(2), 2006
PMID: 16162659
Barton ER., Am J Physiol Cell Physiol 290(2), 2006
PMID: 16162659
20 References
Daten bereitgestellt von Europe PubMed Central.
The mdx-amplification-resistant mutation system assay, a simple and rapid polymerase chain reaction-based detection of the mdx allele.
Amalfitano A, Chamberlain JS., Muscle Nerve 19(12), 1996
PMID: 8941268
Amalfitano A, Chamberlain JS., Muscle Nerve 19(12), 1996
PMID: 8941268
Rapid recovery following contraction-induced injury to in situ skeletal muscles in mdx mice.
Brooks SV., J. Muscle Res. Cell. Motil. 19(2), 1998
PMID: 9536444
Brooks SV., J. Muscle Res. Cell. Motil. 19(2), 1998
PMID: 9536444
The mdx mouse skeletal muscle myopathy: II. Contractile properties.
Coulton GR, Curtin NA, Morgan JE, Partridge TA., Neuropathol. Appl. Neurobiol. 14(4), 1988
PMID: 3221977
Coulton GR, Curtin NA, Morgan JE, Partridge TA., Neuropathol. Appl. Neurobiol. 14(4), 1988
PMID: 3221977
Dp71 can restore the dystrophin-associated glycoprotein complex in muscle but fails to prevent dystrophy.
Cox GA, Sunada Y, Campbell KP, Chamberlain JS., Nat. Genet. 8(4), 1994
PMID: 7894482
Cox GA, Sunada Y, Campbell KP, Chamberlain JS., Nat. Genet. 8(4), 1994
PMID: 7894482
AUTHOR UNKNOWN, 0
Utrophin-dystrophin-deficient mice as a model for Duchenne muscular dystrophy.
Deconinck AE, Rafael JA, Skinner JA, Brown SC, Potter AC, Metzinger L, Watt DJ, Dickson JG, Tinsley JM, Davies KE., Cell 90(4), 1997
PMID: 9288751
Deconinck AE, Rafael JA, Skinner JA, Brown SC, Potter AC, Metzinger L, Watt DJ, Dickson JG, Tinsley JM, Davies KE., Cell 90(4), 1997
PMID: 9288751
Exogenous Dp71 restores the levels of dystrophin associated proteins but does not alleviate muscle damage in mdx mice.
Greenberg DS, Sunada Y, Campbell KP, Yaffe D, Nudel U., Nat. Genet. 8(4), 1994
PMID: 7894483
Greenberg DS, Sunada Y, Campbell KP, Yaffe D, Nudel U., Nat. Genet. 8(4), 1994
PMID: 7894483
Mutual interference of myotonia and muscular dystrophy in the mouse: a study on ADR-MDX double mutants.
Heimann P, Augustin M, Wieneke S, Heising S, Jockusch H., Neuromuscul. Disord. 8(8), 1998
PMID: 10093061
Heimann P, Augustin M, Wieneke S, Heising S, Jockusch H., Neuromuscul. Disord. 8(8), 1998
PMID: 10093061
A 71-kilodalton protein is a major product of the Duchenne muscular dystrophy gene in brain and other nonmuscle tissues.
Lederfein D, Levy Z, Augier N, Mornet D, Morris G, Fuchs O, Yaffe D, Nudel U., Proc. Natl. Acad. Sci. U.S.A. 89(12), 1992
PMID: 1319059
Lederfein D, Levy Z, Augier N, Mornet D, Morris G, Fuchs O, Yaffe D, Nudel U., Proc. Natl. Acad. Sci. U.S.A. 89(12), 1992
PMID: 1319059
Exogenous Dp71 is a dominant negative competitor of dystrophin in skeletal muscle.
Leibovitz S, Meshorer A, Fridman Y, Wieneke S, Jockusch H, Yaffe D, Nudel U., Neuromuscul. Disord. 12(9), 2002
PMID: 12398834
Leibovitz S, Meshorer A, Fridman Y, Wieneke S, Jockusch H, Yaffe D, Nudel U., Neuromuscul. Disord. 12(9), 2002
PMID: 12398834
A new blocking method for application of murine monoclonal antibody to mouse tissue sections.
Lu QL, Partridge TA., J. Histochem. Cytochem. 46(8), 1998
PMID: 9671449
Lu QL, Partridge TA., J. Histochem. Cytochem. 46(8), 1998
PMID: 9671449
Differential expression and subcellular distribution of dystrophin Dp71 isoforms during differentiation process.
Marquez FG, Cisneros B, Garcia F, Ceja V, Velazquez F, Depardon F, Cervantes L, Rendon A, Mornet D, Rosas-vargas H, Mustre M, Montanez C., Neuroscience 118(4), 2003
PMID: 12732241
Marquez FG, Cisneros B, Garcia F, Ceja V, Velazquez F, Depardon F, Cervantes L, Rendon A, Mornet D, Rosas-vargas H, Mustre M, Montanez C., Neuroscience 118(4), 2003
PMID: 12732241
Decreased osmotic stability of dystrophin-less muscle cells from the mdx mouse.
Menke A, Jockusch H., Nature 349(6304), 1991
PMID: 1985268
Menke A, Jockusch H., Nature 349(6304), 1991
PMID: 1985268
AUTHOR UNKNOWN, 0
Dystrophin protects the sarcolemma from stresses developed during muscle contraction.
Petrof BJ, Shrager JB, Stedman HH, Kelly AM, Sweeney HL., Proc. Natl. Acad. Sci. U.S.A. 90(8), 1993
PMID: 8475120
Petrof BJ, Shrager JB, Stedman HH, Kelly AM, Sweeney HL., Proc. Natl. Acad. Sci. U.S.A. 90(8), 1993
PMID: 8475120
Dystrophin and utrophin influence fiber type composition and post-synaptic membrane structure.
Rafael JA, Townsend ER, Squire SE, Potter AC, Chamberlain JS, Davies KE., Hum. Mol. Genet. 9(9), 2000
PMID: 10814717
Rafael JA, Townsend ER, Squire SE, Potter AC, Chamberlain JS, Davies KE., Hum. Mol. Genet. 9(9), 2000
PMID: 10814717
AUTHOR UNKNOWN, 0
Expression of full-length utrophin prevents muscular dystrophy in mdx mice.
Tinsley J, Deconinck N, Fisher R, Kahn D, Phelps S, Gillis JM, Davies K., Nat. Med. 4(12), 1998
PMID: 9846586
Tinsley J, Deconinck N, Fisher R, Kahn D, Phelps S, Gillis JM, Davies K., Nat. Med. 4(12), 1998
PMID: 9846586
AUTHOR UNKNOWN, 0
Generation of tension by skinned fibers and intact skeletal muscles from desmin-deficient mice.
Wieneke S, Stehle R, Li Z, Jockusch H., Biochem. Biophys. Res. Commun. 278(2), 2000
PMID: 11097852
Wieneke S, Stehle R, Li Z, Jockusch H., Biochem. Biophys. Res. Commun. 278(2), 2000
PMID: 11097852
Export
Markieren/ Markierung löschen
Markierte Publikationen
Web of Science
Dieser Datensatz im Web of Science®Quellen
PMID: 14555666
PubMed | Europe PMC
Suchen in