DOI: 10.1083/jcb.152.5.1107. Deletion of Murine <i>SMN</i> Exon 7 Directed to Skeletal Muscle Leads to Severe Muscular Dystrophy

Deletion of Murine SMN Exon 7 Directed to Skeletal Muscle Leads to Severe Muscular Dystrophy

10.1083/jcb.152.5.1107

Crossref journal-article

Rockefeller University Press

The Journal of Cell Biology (291)

Abstract

Spinal muscular atrophy (SMA) is characterized by degeneration of motor neurons of the spinal cord associated with muscle paralysis and caused by mutations of the survival motor neuron gene (SMN). To determine whether SMN gene defect in skeletal muscle might have a role in SMA pathogenesis, deletion of murine SMN exon 7, the most frequent mutation found in SMA, has been restricted to skeletal muscle by using the Cre-loxP system. Mutant mice display ongoing muscle necrosis with a dystrophic phenotype leading to muscle paralysis and death. The dystrophic phenotype is associated with elevated levels of creatine kinase activity, Evans blue dye uptake into muscle fibers, reduced amount of dystrophin and upregulation of utrophin expression suggesting a destabilization of the sarcolemma components. The mutant mice will be a valuable model for elucidating the underlying mechanism. Moreover, our results suggest a primary involvement of skeletal muscle in human SMA, which may contribute to motor defect in addition to muscle denervation caused by the motor neuron degeneration. These data may have important implications for the development of therapeutic strategies in SMA.

Bibliography

Cifuentes-Diaz, C., Frugier, T., Tiziano, F. D., LacÃ¨ne, E., Roblot, N., Joshi, V., Moreau, M. H., & Melki, J. (2001). Deletion of Murine SMN Exon 7 Directed to Skeletal Muscle Leads to Severe Muscular Dystrophy. The Journal of Cell Biology, 152(5), 1107â1114.

Authors 8

Carmen Cifuentes-Diaz (first)
Tony Frugier (additional)
Francesco D. Tiziano (additional)
Emmanuelle Lacène (additional)
Natacha Roblot (additional)
Vandana Joshi (additional)
Marie Helene Moreau (additional)
Judith Melki (additional)

References 25 Referenced 190

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10.1016/0022-510X(80)90136-7 / J. Neurol. Sci. / Spinal muscular atrophy and hypertrophy of the calves by Bouwsma (1980)
10.1016/S0140-6736(95)90869-2 / Lancet. / Constitutive muscular abnormalities in culture in spinal muscular atrophy by Braun (1995)
10.1073/pnas.81.4.1189 / Proc. Natl. Acad. Sci. USA. / X chromosome-linked muscular dystrophy (mdx) in the mouse by Bulfield (1984)
10.1083/jcb.142.6.1461 / J. Cell Biol. / Progressive muscular dystrophy in alpha-sarcoglycan-deficient mice by Duclos (1998)
10.1016/S1097-2765(00)80410-4 / Mol. Cell. / Disruption of the beta-sarcoglycan gene reveals pathogenetic complexity of limb-girdle muscular dystrophy type 2E by Durbeej (2000)
10.1016/S0092-8674(00)80368-2 / Cell. / The SMN-SIP1 complex has an essential role in spliceosomal snRNP biogenesis by Fischer (1997)
10.1093/hmg/9.5.849 / Hum. Mol. Genet. / Nuclear targeting defect of SMN lacking the C-terminus in a mouse model of spinal muscular atrophy by Frugier (2000)
10.1212/WNL.37.8.1361 / Neurology / Extracts of muscle biopsies from patients with spinal muscular atrophies inhibit neurite outgrowth from spinal neurons by Henderson (1987)
10.1038/71709 / Nat. Genet. / A mouse model for spinal muscular atrophy by Hsieh-Li (2000)
10.1172/JCI3705 / J. Clin. Invest. / Merosin-deficient congenital muscular dystrophy. Partial genetic correction in two mouse models by Kuang (1998)
{'key': '2023072211302983700_KugelbergandWelander1956', 'first-page': '500', 'article-title': 'Heredofamilial juvenile muscular atrophy simulating muscular dystrophy', 'volume': '75', 'author': 'Kugelberg', 'year': '1956', 'journal-title': 'Acta Neurol. Psychiatr.'} / Acta Neurol. Psychiatr. / Heredofamilial juvenile muscular atrophy simulating muscular dystrophy by Kugelberg (1956)
10.1016/0092-8674(95)90460-3 / Cell / Identification and characterization of a spinal muscular atrophy-determining gene by Lefebvre (1995)
10.1002/j.1460-2075.1996.tb00725.x / EMBO (Eur. Mol. Biol. Organ.) J. / A novel nuclear structure containing the survival of motor neuron proteins by Liu (1996)
10.1016/0022-510X(71)90249-8 / J. Neurol. Sci. / Histological and histochemical changes from cases of chronic juvenile and early adult spinal muscular atrophy (the Kugelberg-Welander syndrome) by Mastaglia (1971)
10.1097/00019052-199710000-00005 / Curr. Opin. Neurol. / Spinal muscular atrophy by Melki (1997)
10.1093/nar/27.19.e27 / Nucleic Acids Res. / Gene targeting restricted to mouse striated muscle lineage by Miniou (1999)
10.1093/hmg/9.3.333 / Hum. Mol. Genet. / The human centromeric Survival Motor neuron gene (SMN2) rescues embryonic lethality in SMN−/− mice and results in a mouse with spinal muscular atrophy by Monani (2000)
10.1016/0960-8966(91)90052-T / Neuromusc. Disord. / Workshop reportinternational SMA collaboration by Munsat (1991)
10.1016/0022-510X(70)90001-8 / J. Neurol. Sci. / Chronic spinal muscular atrophy by Namba (1970)
10.1016/S0092-8674(00)81632-3 / Cell. / A novel function for SMN, the spinal muscular atrophy disease gene product, in pre-mRNA splicing by Pellizzoni (1998)
10.1073/pnas.85.14.5166 / Proc. Natl. Acad. Sci. USA. / Site-specific DNA recombination in mammalian cells by the Cre recombinase of bacteriophage P1 by Sauer (1988)
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10.1016/0022-2836(81)90375-2 / J. Mol. Biol. / Bacteriophage P1 site-specific recombination. I. Recombination between loxP sites by Sternberg (1981)

Dates

Type	When
Created	23 years, 1 month ago (July 26, 2002, 12:46 p.m.)
Deposited	2 years, 1 month ago (July 22, 2023, 7:30 a.m.)
Indexed	1 month ago (Aug. 3, 2025, 12:22 a.m.)
Issued	24 years, 5 months ago (March 5, 2001)
Published	24 years, 5 months ago (March 5, 2001)
Published Online	24 years, 5 months ago (March 5, 2001)
Published Print	24 years, 5 months ago (March 5, 2001)

Funders 0

None

BibTeX

@article{Cifuentes_Diaz_2001, title={Deletion of Murine SMN Exon 7 Directed to Skeletal Muscle Leads to Severe Muscular Dystrophy}, volume={152}, ISSN={1540-8140}, url={http://dx.doi.org/10.1083/jcb.152.5.1107}, DOI={10.1083/jcb.152.5.1107}, number={5}, journal={The Journal of Cell Biology}, publisher={Rockefeller University Press}, author={Cifuentes-Diaz, Carmen and Frugier, Tony and Tiziano, Francesco D. and Lacène, Emmanuelle and Roblot, Natacha and Joshi, Vandana and Moreau, Marie Helene and Melki, Judith}, year={2001}, month=mar, pages={1107–1114} }

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  "journal-issue": {
    "issue": "5",
    "published-print": {
      "date-parts": [
        [
          2001,
          3,
          5
        ]
      ]
    }
  },
  "URL": "http://dx.doi.org/10.1083/jcb.152.5.1107",
  "relation": {},
  "ISSN": [
    "0021-9525",
    "1540-8140"
  ],
  "subject": [],
  "published": {
    "date-parts": [
      [
        2001,
        3,
        5
      ]
    ]
  }
}