Abstract
Duodenal smooth muscle cells of normal scn mice and dystrophin-deficient mdx mice were prepared by the in vivo cryotechnique (IVCT). The IVCT revealed more realistic ultrastructures of functional smooth muscle cells in both scn and mdx mice. In the scn mice, they were surrounded by compact extracellular matrix and had large gap junctions and closed caveolae with electron-dense contents. In the dystrophic mdx mice, however, the extracellular matrix was often torn off from the surface of the smooth muscle cells. The dystrophic mdx mouse had smooth muscle cells equipped with small gap junctions and uneven dense plaques in comparison with the normal scn mouse. They were surrounded with some incomplete basal lamina. The dystrophic smooth muscle cells also represented the atypical arrangement of caveolae, which were fusing together with neighboring caveolae. Such ultrastructural changes were mainly seen in the regions of caveolae, suggesting that a primary cause of the smooth muscle injury might be induced by the lack of normal arrangements of functional cell membranes.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Carlson CG (1998) The dystrophinopathies: an alternative to the structural hypothesis. Neurobiol Dis 5:3–15
Sicinski P, Allan YG, Ryder-Cook AS, Barnard EA, Darlison MG, Barnard PJ (1989) The molecular basis of muscular dystrophy in the mdx mouse: a point mutation. Science 244:1578–1580
Harricane MC, Augier N, Leger J, Anoal M, Cavadore C, Mornet D (1991) Ultrastructural localization of dystrophin in chicken smooth muscle. Cell Biol Intern Rep 15:687–697
Marbini A, Gemignani F, Bellanova MF, Guidetti D, Ferrari A (1996) Immunohistochemical localization of utrophin and other cytoskeletal proteins in skin smooth muscle in neuromuscular diseases. J Neurol Sci 143:156–160
Marbini A, Marcello N, Bellanova MF, Guidetti D, Ferrari A, Gemignani F (1995) Dystrophin expression in skin biopsy immunohistochemical localisation of striated muscle type dystrophin. J Neurol Sci 129:29–33
Byers TJ, Kunkel LM, Watkins SC (1991) The subcellular distribution of dystrophin in mouse skeletal, cardiac, and smooth muscle. J Cell Biol 115:411–421
Brown RH Jr (1997) Dystrophin-associated proteins and the muscular dystrophies. Annu Rev Med 48:457–466
Pasternak C, Wong S, Elson EL (1995) Mechanical function of dystrophin in muscle cells. J Cell Biol 128:355–361
Gabella G (1981) Structure of smooth muscle. In: Buelbring E, Brading AF, Jones AW, Tomita T (eds) An assessment of current knowledge. University of Texas Press, Austin, pp 1–46
North AJ, Galazkiewicz B, Byers TJ, Glenney JR Jr, Small JV (1993) Complementary distributions of vinculin and dystrophin define two distinct sarcolemma domains in smooth muscle. J Cell Biol 120:1159–1167
Okamoto T, Schlegel A, Scherer PE, Lisanti MP (1998) Caveolins, a family of scaffolding proteins for organizing “preassembled signaling complexes” at the plasma membrane. J Biol Chem 273:5419–5422
Ohno S, Terada N, Fujii Y, Ueda H, Takayama I (1996) Dynamic structure of glomerular capillary loop as revealed by an in vivo cryotechnique. Virchows Arch 427:519–527
McCarter GC, Denetclaw WFJ, Reddy P, Steinhardt RA (1997) Lipofection of a cDNA plasmid containing the dystrophin gene lowers intracellular free calcium and calcium leak channel activity in mdx myotubes. Gene Ther 4:483–487
Boland B, Himpens B, Casteels R, Gillis JM (1993) Lack of dystrophin but normal calcium homeostasis in smooth muscle from dystrophic mdx mice. J Muscle Res Cell Motil 14:133–139
Khurana TS, Prendergast RA, Alameddine HS, Tomé FM, Fardeau M, Arahata K, Sugita H, Kunkel LM (1995) Absence of extraocular muscle pathology in Duchenne’s muscular dystrophy: role for calcium homeostasis in extraocular muscle sparing. J Exp Med 182:467–475
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2016 Springer Japan
About this chapter
Cite this chapter
Ohno, S. (2016). Dynamic Ultrastructure of Smooth Muscle Cells in Dystrophin-Deficient mdx or Normal scn Mice. In: Ohno, S., Ohno, N., Terada, N. (eds) In Vivo Cryotechnique in Biomedical Research and Application for Bioimaging of Living Animal Organs. Springer, Tokyo. https://doi.org/10.1007/978-4-431-55723-4_13
Download citation
DOI: https://doi.org/10.1007/978-4-431-55723-4_13
Publisher Name: Springer, Tokyo
Print ISBN: 978-4-431-55722-7
Online ISBN: 978-4-431-55723-4
eBook Packages: MedicineMedicine (R0)