Difference between revisions of "File:Dystrophin in the muscle fibre membrane.jpg"

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(This image is a visual display of the muscle fibre membrane and the location of dystrophin within the sarcolemma surrounding each muscle fibre. {{Template:2011 Student Image}}. Image creator: Ashleigh Pontifex (z3332629) Student image constructed bas)
 
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There are 4 main transmembrane proteins that compose the dystrophin-glycoprotein complex (DGP), also referred to as the dystrophin-associated protein complex (DAPC), located in the muscle fibre membrane. These proteins include:
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* '''Sarcoglycans:''' a family of transmembrane proteins that work in conjunction with other proteins (namely dystrophin) to protect muscle fibres during contraction. There are 4 main types present in striated muscle, including: α-sarcoglycan, β-sarcoglycan, γ-sarcoglycan and δ-sarcoglycan. The sarcoglycan complex is disrupted and destabilized from the plasma membrane when dystrophin is mutated and hence leads to eccentric contraction-induced disruption of the plasma membrane of smooth and cardiac muscle. <ref>Hack, A.A., Lam, M, J.,Cordier, L., Shoturma, D.I (2000). “Differential requirement for individual sarcoglycans and dystrophin in the assembly and function of the dystrophin-glycoprotein complex”. Journal of Cell Science 113, page 2535-2544 (2000). Accessed via: http://jcs.biologists.org/content/113/14/2535.full.pdf</ref>
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* '''Dystroglycan:''' connects the extracellular matrix and cytoskeleton and also forms a linkage between the dystrophin-glycoprotein complex and the basal lamina. When dystrophin is mutated, these linkages formed by dystroglycan are lost and also cause the disruption of the plasma membrane.<ref>Neuromuscular (2011). “Membrane-associated protein complexes in skeletal muscle fibres and connective tissue.” Author anonymous. Accessed via: http://neuromuscular.wustl.edu/musdist/dag2.htm#ad</ref>
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* '''Syntrophin:''' links to the extracellular matrix through dystrophin and creates signal transduction complexes at the DAPC and therefore when dystrophin is mutated, this crucial signalling pathway is lost. <ref>Davies.K.E, Nowak. K.J (2006). "Molecular mechanisms of musclar dystrophies: old and new players." Nature Reviews. October, 2006 Volume 7, pages 763-773.</ref>
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* '''Dystrobrevinis:''' the functional role of this complex has not yet been clearly identified, however studies show that dystrobrevin disappears from the muscle membrane in Duchenne muscular dystrophy (DMD). <ref>Grisoni,K., Gieseler,K., Se´ galat,L. (2002). “Dystrobrevin requires a dystrophin-binding domain to function in Caenorhabditis elegans”. Eur. J. Biochem. 269, pages 1607±1612.</ref>
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Image creator: Ashleigh Pontifex (z3332629)
 
Image creator: Ashleigh Pontifex (z3332629)

Revision as of 12:49, 6 October 2011

This image is a visual display of the muscle fibre membrane and the location of dystrophin within the sarcolemma surrounding each muscle fibre.


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Cite this page: Hill, M.A. (2021, July 29) Embryology Dystrophin in the muscle fibre membrane.jpg. Retrieved from https://embryology.med.unsw.edu.au/embryology/index.php/File:Dystrophin_in_the_muscle_fibre_membrane.jpg

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There are 4 main transmembrane proteins that compose the dystrophin-glycoprotein complex (DGP), also referred to as the dystrophin-associated protein complex (DAPC), located in the muscle fibre membrane. These proteins include:

  • Sarcoglycans: a family of transmembrane proteins that work in conjunction with other proteins (namely dystrophin) to protect muscle fibres during contraction. There are 4 main types present in striated muscle, including: α-sarcoglycan, β-sarcoglycan, γ-sarcoglycan and δ-sarcoglycan. The sarcoglycan complex is disrupted and destabilized from the plasma membrane when dystrophin is mutated and hence leads to eccentric contraction-induced disruption of the plasma membrane of smooth and cardiac muscle. [1]
  • Dystroglycan: connects the extracellular matrix and cytoskeleton and also forms a linkage between the dystrophin-glycoprotein complex and the basal lamina. When dystrophin is mutated, these linkages formed by dystroglycan are lost and also cause the disruption of the plasma membrane.[2]
  • Syntrophin: links to the extracellular matrix through dystrophin and creates signal transduction complexes at the DAPC and therefore when dystrophin is mutated, this crucial signalling pathway is lost. [3]
  • Dystrobrevinis: the functional role of this complex has not yet been clearly identified, however studies show that dystrobrevin disappears from the muscle membrane in Duchenne muscular dystrophy (DMD). [4]


Image creator: Ashleigh Pontifex (z3332629)

Student image constructed based on the image presented on: http://www.mda.org/publications/fa-dmdbmd-what.html

Location of dystrophin within the muscle fibre membrane by Ashleigh Pontifex is licensed under a Creative Commons Attribution-NoDerivs 3.0 Unported License. Based on a work at www.mda.org.

<a rel="license" href="http://creativecommons.org/licenses/by-nd/3.0/"><img alt="Creative Commons License" style="border-width:0" src="http://i.creativecommons.org/l/by-nd/3.0/88x31.png" /></a>
Location of dystrophin within the muscle fibre membrane by Ashleigh Pontifex is licensed under a <a rel="license" href="http://creativecommons.org/licenses/by-nd/3.0/">Creative Commons Attribution-NoDerivs 3.0 Unported License</a>.
Based on a work at <a xmlns:dct="http://purl.org/dc/terms/" href="http://www.mda.org/publications/fa-dmdbmd-what.html" rel="dct:source">www.mda.org</a>.

  1. Hack, A.A., Lam, M, J.,Cordier, L., Shoturma, D.I (2000). “Differential requirement for individual sarcoglycans and dystrophin in the assembly and function of the dystrophin-glycoprotein complex”. Journal of Cell Science 113, page 2535-2544 (2000). Accessed via: http://jcs.biologists.org/content/113/14/2535.full.pdf
  2. Neuromuscular (2011). “Membrane-associated protein complexes in skeletal muscle fibres and connective tissue.” Author anonymous. Accessed via: http://neuromuscular.wustl.edu/musdist/dag2.htm#ad
  3. Davies.K.E, Nowak. K.J (2006). "Molecular mechanisms of musclar dystrophies: old and new players." Nature Reviews. October, 2006 Volume 7, pages 763-773.
  4. Grisoni,K., Gieseler,K., Se´ galat,L. (2002). “Dystrobrevin requires a dystrophin-binding domain to function in Caenorhabditis elegans”. Eur. J. Biochem. 269, pages 1607±1612.

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