Muscle fibers from dysgenic mouse in vivo lack a surface component of peripheral couplings

Franzini‐Armstrong, Clara; Pinçon‐Raymond, Martine; Rieger, François

doi:10.1016/0012-1606(91)90238-x

Cited by 79 publications

(59 citation statements)

References 52 publications

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“…1G). Similar abnormalities of the skeletal muscle development and organization were detected previously in the dysgenic (21,35) and dyspedic mouse models (22,36), suggesting that similar intracellular pathways might be affected in all three models. Skeletal Abnormalities.…”

Section: Resultssupporting

confidence: 77%

An Ryr1 ^I4895T mutation abolishes Ca ²⁺ release channel function and delays development in homozygous offspring of a mutant mouse line

Zvaritch

Depreux

Kraeva

et al. 2007

Proc. Natl. Acad. Sci. U.S.A.

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A heterozygous Ile4898 to Thr (I4898T) mutation in the human type 1 ryanodine receptor/Ca 2+ release channel (RyR1) leads to a severe form of central core disease. We created a mouse line in which the corresponding Ryr1 I4895T mutation was introduced by using a “knockin” protocol. The heterozygote does not exhibit an overt disease phenotype, but homozygous (IT/IT) mice are paralyzed and die perinatally, apparently because of asphyxia. Histological analysis shows that IT/IT mice have greatly reduced and amorphous skeletal muscle. Myotubes are small, nuclei remain central, myofibrils are disarranged, and no cross striation is obvious. Many areas indicate probable degeneration, with shortened myotubes containing central stacks of pyknotic nuclei. Other manifestations of a delay in completion of late stages of embryogenesis include growth retardation and marked delay in ossification, dermatogenesis, and cardiovascular development. Electron microscopy of IT/IT muscle demonstrates appropriate targeting and positioning of RyR1 at triad junctions and a normal organization of dihydropyridine receptor (DHPR) complexes into RyR1-associated tetrads. Functional studies carried out in cultured IT/IT myotubes show that ligand-induced and DHPR-activated RyR1 Ca 2+ release is absent, although retrograde enhancement of DHPR Ca 2+ conductance is retained. IT/IT mice, in which RyR1-mediated Ca 2+ release is abolished without altering the formation of the junctional DHPR-RyR1 macromolecular complex, provide a valuable model for elucidation of the role of RyR1-mediated Ca 2+ signaling in mammalian embryogenesis.

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Section: Resultssupporting

confidence: 77%

An Ryr1 ^I4895T mutation abolishes Ca ²⁺ release channel function and delays development in homozygous offspring of a mutant mouse line

Zvaritch

Depreux

Kraeva

et al. 2007

Proc. Natl. Acad. Sci. U.S.A.

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“…412, asterisks); random distribution of intramembranous particles of variable sizes; and presence of junctional tetrads at peripheral couplings (Fig. 4b,c, semicircles, see Franzini-Armstrong et al, 1991). Endothelial cells also have caveolae, but their intramembranous particles are far more uniform in size, and tight junctions are often visible.…”

Section: Quantitation Of Gap Junctionsmentioning

confidence: 97%

Differences in the histogenesis of EDL and diaphragm in rat

Yiping

Appelt

Kelly

et al. 1992

Developmental Dynamics

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We have examined the histogenesis of the diaphragm and extensor digitorum muscle in rat embryos, with the aim of defining differences in developmental patterns that can be related to the functional requirements of these muscles during and after development.Patterns of interactions between myotubes and other cells, and frequency of gap junctions are quite different in the two muscles. In diaphragm, primary myotubes (at day 16 in utero) are closely associated with each other, forming parallel sheets or palisades and communicating by gap junctions. Secondary myotubes have formed by day 18, but are immature, and the frequency of gap junctions is lower. The arrangement in palisades is maintained even after fibers are separated from each other by their individual basal lamina. In EDL primary fibers at day 16 have fewer gap junctions, and the peak in communication occurs after the appearance of secondary myotubes (day 18 and 21). Secondary myotubes are more mature than in diaphragm at day 18. 0 1992 Wiley-Liss, Inc.

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“…Surprisingly, absence of either α 1 sDHPR or RyR1 does not affect either formation of junctions or targeting of the second protein to junctional domains. In dysgenic muscle, triads containing organized arrays of feet are formed even if DHPRs are missing (33), and in dyspedic mice both triads and peripheral couplings are formed in the absence of RyRs (34). In regards to the targeting of DHPRs to junctions lacking RyRs, there have been some controversial results.…”

Section: Dysgenic and Dyspedic Mice Contribution To The Understandingmentioning

confidence: 99%

Structural interaction between RYRs and DHPRs in calcium release units of cardiac and skeletal muscle cells

Protasi¹

2002

Front Biosci

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Muscle fibers from dysgenic mouse in vivo lack a surface component of peripheral couplings

Cited by 79 publications

References 52 publications

An Ryr1 ^I4895T mutation abolishes Ca ²⁺ release channel function and delays development in homozygous offspring of a mutant mouse line

An Ryr1 ^I4895T mutation abolishes Ca ²⁺ release channel function and delays development in homozygous offspring of a mutant mouse line

Differences in the histogenesis of EDL and diaphragm in rat

Structural interaction between RYRs and DHPRs in calcium release units of cardiac and skeletal muscle cells

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Muscle fibers from dysgenic mouse in vivo lack a surface component of peripheral couplings

Cited by 79 publications

References 52 publications

An Ryr1 I4895T mutation abolishes Ca 2+ release channel function and delays development in homozygous offspring of a mutant mouse line

An Ryr1 I4895T mutation abolishes Ca 2+ release channel function and delays development in homozygous offspring of a mutant mouse line

Differences in the histogenesis of EDL and diaphragm in rat

Structural interaction between RYRs and DHPRs in calcium release units of cardiac and skeletal muscle cells

Contact Info

Product

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An Ryr1 ^I4895T mutation abolishes Ca ²⁺ release channel function and delays development in homozygous offspring of a mutant mouse line

An Ryr1 ^I4895T mutation abolishes Ca ²⁺ release channel function and delays development in homozygous offspring of a mutant mouse line