Stages in fibre breakdown in duchenne muscular dystrophy

Cullen, Michael J.; Fulthorpe, J. J.

doi:10.1016/0022-510x(75)90232-4

Cited by 150 publications

(49 citation statements)

References 12 publications

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“…Consistent with our findings is that both hypercontraction and overstretching of sarcomeres is seen in vertebrate dystrophic muscles before they progress to a more advanced degeneration phenotype (Cullen and Fulthorpe, 1975). A tendency to hypercontract is also associated with disruption of DGC proteins in Caenorhabditis elegans (Bessou et al, 1998;Grisoni et al, 2002).…”

Section: Discussionsupporting

confidence: 78%

Dystroglycan and ProteinO-Mannosyltransferases 1 and 2 Are Required to Maintain Integrity ofDrosophilaLarval Muscles

Haines

Seabrooke

Stewart

2007

MBoC

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In vertebrates, mutations in Protein O-mannosyltransferase1 (POMT1) or POMT2 are associated with muscular dystrophy due to a requirement for O-linked mannose glycans on the Dystroglycan (Dg) protein. In this study we examine larval body wall muscles of Drosophila mutant for Dg, or RNA interference knockdown for Dg and find defects in muscle attachment, altered muscle contraction, and a change in muscle membrane resistance. To determine if POMTs are required for Dg function in Drosophila, we examine larvae mutant for genes encoding POMT1 or POMT2. Larvae mutant for either POMT, or doubly mutant for both, show muscle attachment and muscle contraction phenotypes identical to those associated with reduced Dg function, consistent with a requirement for O-linked mannose on Drosophila Dg. Together these data establish a central role for Dg in maintaining integrity in Drosophila larval muscles and demonstrate the importance of glycosylation to Dg function in Drosophila. This study opens the possibility of using Drosophila to investigate muscular dystrophy.

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Section: Discussionsupporting

confidence: 78%

Dystroglycan and ProteinO-Mannosyltransferases 1 and 2 Are Required to Maintain Integrity ofDrosophilaLarval Muscles

Haines

Seabrooke

Stewart

2007

MBoC

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“…Interestingly, some dystrophic muscles show hyper-contractility during early stages of their degeneration (Cullen and Fulthorpe, 1975;Gupta et al, 2012;Haines et al, 2007). We hypothesized, therefore, that the eggs with an increased aspect ratio might have developed within a hyper-contractile muscle sheath.…”

Section: Resultsmentioning

confidence: 99%

Influence of ovarian muscle contraction and oocyte growth on egg chamber elongation in Drosophila

Andersen

Horne‐Badovinac

2016

Development

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Organs are formed from multiple cell types that make distinct contributions to their shape. The Drosophila egg chamber provides a tractable model to dissect such contributions during morphogenesis. Egg chambers consist of 16 germ cells (GCs) surrounded by a somatic epithelium. Initially spherical, these structures elongate as they mature. This morphogenesis is thought to occur through a 'molecular corset' mechanism, whereby structural elements within the epithelium become circumferentially organized perpendicular to the elongation axis and resist the expansive growth of the GCs to promote elongation. Whether this epithelial organization provides the hypothesized constraining force has been difficult to discern, however, and a role for GC growth has not been demonstrated. Here, we provide evidence for this mechanism by altering the contractile activity of the tubular muscle sheath that surrounds developing egg chambers. Muscle hypo-contraction indirectly reduces GC growth and shortens the egg, which demonstrates the necessity of GC growth for elongation. Conversely, muscle hyper-contraction enhances the elongation program. Although this is an abnormal function for this muscle, this observation suggests that a corset-like force from the egg chamber's exterior could promote its lengthening. These findings highlight how physical contributions from several cell types are integrated to shape an organ.

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“…In DMD patients 9,11,13,34 and mdx mice, 41 necrotic muscle fibers, rounded (hypercontracted) muscle fibers, and fibers with disorganized Z lines 13,41 or the absence of Z lines 11 are frequently observed. These rounded fibers accumulate calcium.…”

Section: Discussionmentioning

confidence: 99%

“…In DMD patients 9,11,13,34 and mdx mice, 41 rounded muscle fibers were disorganized, 13,41 or lacked Z lines. 11 Accumulation of calcium was also found in these fibers.…”

mentioning

confidence: 99%

Skeletal muscle fiber degeneration in mdx mice induced by electrical stimulation

et al. 1997

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Stages in fibre breakdown in duchenne muscular dystrophy

Cited by 150 publications

References 12 publications

Dystroglycan and ProteinO-Mannosyltransferases 1 and 2 Are Required to Maintain Integrity ofDrosophilaLarval Muscles

Dystroglycan and ProteinO-Mannosyltransferases 1 and 2 Are Required to Maintain Integrity ofDrosophilaLarval Muscles

Influence of ovarian muscle contraction and oocyte growth on egg chamber elongation in Drosophila

Skeletal muscle fiber degeneration in mdx mice induced by electrical stimulation

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