scholarly journals Analysis of the dystrophin, muscular dystrophy (Dmd)-dependent transcriptome in mouse diaphragm muscle

2019 ◽  
Author(s):  
JD Porter
Keyword(s):  
Muscular Dystrophy ◽  
Diaphragm Muscle ◽  
Mouse Diaphragm

scholarly journals Mass Spectrometric Profiling of Extraocular Muscle and Proteomic Adaptations in the mdx-4cv Model of Duchenne Muscular Dystrophy

Life ◽  
2021 ◽  
Vol 11 (7) ◽  
pp. 595
Author(s):  
Stephen Gargan ◽  
Paul Dowling ◽  
Margit Zweyer ◽  
Jens Reimann ◽  
Michael Henry ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Biochemical Properties ◽  
Mass Spectrometric ◽  
Cellular Stress Response ◽  
Diaphragm Muscle ◽  
Spectrometric Analysis ◽  
Mitochondrial Enzymes ◽  
Wild Type ◽  
Potential Marker

Extraocular muscles (EOMs) represent a specialized type of contractile tissue with unique cellular, physiological, and biochemical properties. In Duchenne muscular dystrophy, EOMs stay functionally unaffected in the course of disease progression. Therefore, it was of interest to determine their proteomic profile in dystrophinopathy. The proteomic survey of wild type mice and the dystrophic mdx-4cv model revealed a broad spectrum of sarcomere-associated proteoforms, including components of the thick filament, thin filament, M-band and Z-disk, as well as a variety of muscle-specific markers. Interestingly, the mass spectrometric analysis revealed unusual expression levels of contractile proteins, especially isoforms of myosin heavy chain. As compared to diaphragm muscle, both proteomics and immunoblotting established isoform MyHC14 as a new potential marker in wild type EOMs, in addition to the previously identified isoforms MyHC13 and MyHC15. Comparative proteomics was employed to establish alterations in the protein expression profile between normal EOMs and dystrophin-lacking EOMs. The analysis of mdx-4cv EOMs identified elevated levels of glycolytic enzymes and molecular chaperones, as well as decreases in mitochondrial enzymes. These findings suggest a process of adaptation in dystrophin-deficient EOMs via a bioenergetic shift to more glycolytic metabolism, as well as an efficient cellular stress response in EOMs in dystrophinopathy.

scholarly journals Developmental regulation of membrane traffic organization during synaptogenesis in mouse diaphragm muscle.

1995 ◽  
Vol 130 (4) ◽  
pp. 959-968 ◽  
Author(s):  
C Antony ◽  
M Huchet ◽  
J P Changeux ◽  
J Cartaud
Keyword(s):  
Secretory Pathway ◽  
Developmental Regulation ◽  
Membrane Traffic ◽  
Synaptic Proteins ◽  
Diaphragm Muscle ◽  
Mouse Diaphragm ◽  
Immunocytochemical Techniques ◽  
Basic Functions ◽  
Traffic Organization ◽  
Exocytic Pathway

In innervated adult skeletal muscles, the Golgi apparatus (GA) displays a set of remarkable features in comparison with embryonic myotubes. We have previously shown by immunocytochemical techniques, that in adult innervated fibers, the GA is no longer associated with all the nuclei, but appears to be concentrated mostly in the subneural domain under the nerve endings in chick (Jasmin, B. J., J. Cartaud, M. Bornens, and J.-P. Changeux. 1989. Proc. Natl. Acad. Sci. USA. 86:7218-7222) and rat (Jasmin, B. J., C. Antony, J.-P. Changeux, and J. Cartaud. 1995. Eur. J. Neurosci. 7:470-479). In addition to such compartmentalization, biochemical modifications take place that suggest a functional specialization of the subsynaptic GA. Here, we focused on the developmental regulation of the membrane traffic organization during the early steps of synaptogenesis in mouse diaphragm muscle. We investigated by immunofluorescence microscopy on cryosections, the distribution of selected subcompartments of the exocytic pathway, and also of a representative endocytic subcompartment with respect to the junctional or extrajunctional domains of developing myofibers. We show that throughout development the RER, the intermediate compartment, and the prelysosomal compartment (mannose 6-phosphate receptor-rich compartment) are homogeneously distributed along the fibers, irrespective of the subneural or extrajunctional domains. In contrast, at embryonic day E17, thus 2-3 d after the onset of innervation, most GA markers become restricted to the subneural domain. Interestingly, some Golgi markers (e.g., alpha-mannosidase II, TGN 38, present in the embryonic myotubes) are no longer detected in the innervated fiber even in the subsynaptic GA. These data show that in innervated muscle fibers, the distal part of the biosynthetic pathway, i.e., the GA, is remodeled selectively shortly after the onset of innervation. As a consequence, in the innervated fiber, the GA exists both as an evenly distributed organelle with basic functions, and as a highly differentiated subsynaptic organelle ensuring maturation and targeting of synaptic proteins. Finally, in the adult, denervation of a hemidiaphragm causes a burst of reexpression of all Golgi markers in extrasynaptic domains of the fibers, hence showing that the particular organization of the secretory pathway is placed under nerve control.

The mdx mouse diaphragm reproduces the degenerative changes of Duchenne muscular dystrophy

Nature ◽  
1991 ◽  
Vol 352 (6335) ◽  
pp. 536-539 ◽  
Author(s):  
H. H. Stedman ◽  
H. L. Sweeney ◽  
J. B. Shrager ◽  
H. C. Maguire ◽  
R. A. Panettieri ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Degenerative Changes ◽  
Mdx Mouse ◽  
Mouse Diaphragm

scholarly journals Neurovascular alignment in mouse diaphragm muscle

2007 ◽  
Vol 21 (5) ◽  
Author(s):  
Diego Correa ◽  
Steven S. Segal
Keyword(s):  
Diaphragm Muscle ◽  
Mouse Diaphragm
Sign in / Sign up

Export Citation Format

Share Document