Deficiency of alpha-sarcoglycan differently affects fast-and slow-twitch skeletal muscles. (Articolo in rivista)

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  • Deficiency of alpha-sarcoglycan differently affects fast-and slow-twitch skeletal muscles. (Articolo in rivista) (literal)
Anno
  • 2005-01-01T00:00:00+01:00 (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#doi
  • 10.1152/ajpregu.00673.2004. (literal)
Alternative label
  • Danieli Betto D, Esposito A, Germinario E, Sandona D, Martinello T, Jakubiec-Puka A, Biral D, Betto R (2005)
    Deficiency of alpha-sarcoglycan differently affects fast-and slow-twitch skeletal muscles.
    in American journal of physiology. Regulatory, integrative and comparative physiology
    (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori
  • Danieli Betto D, Esposito A, Germinario E, Sandona D, Martinello T, Jakubiec-Puka A, Biral D, Betto R (literal)
Pagina inizio
  • 1328 (literal)
Pagina fine
  • 1337 (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#numeroVolume
  • 289 (literal)
Rivista
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#pagineTotali
  • 10 (literal)
Note
  • ISI Web of Science (WOS) (literal)
Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni
  • Danieli Betto D, Esposito A, Germinario E: Dipartimento di Anatomia e Fisiologia Umana, Universit√† di Padova Sandona D, Martinello T: Dipartimento di Scienze Biomediche, Universit√† di Padova Jakubiec-Puka A: Department of Cell Biochemistry, Nencki Institute of Experimental Biology, Warszawa, Poland (literal)
Titolo
  • Deficiency of alpha-sarcoglycan differently affects fast-and slow-twitch skeletal muscles. (literal)
Abstract
  • alpha-Sarcoglycan (Sgca) is a transmembrane glycoprotein of the dystrophin complex located at skeletal and cardiac muscle sarcolemma. Defects in the alpha-sarcoglycan gene (Sgca) cause the severe human-type 2D limb girdle muscular dystrophy. Because Sgca-null mice develop progressive muscular dystrophy similar to human disorder they are a valuable animal model for investigating the physiopathology of the disorder. In this study, biochemical and functional properties of fast-twitch extensor digitorum longus (EDL) and slow-twitch soleus muscles of the Sgca-null mice were analyzed. EDL muscle of Sgcanull mice showed twitch and tetanic kinetics comparable with those of wild-type controls. In contrast, soleus muscle showed reduction of twitch half-relaxation time, prolongation of tetanic half-relaxation time, and increase of maximal rate of rise of tetanus. EDL muscle of Sgca-null mice demonstrated a marked reduction of specific twitch and tetanic tensions and a higher resistance to fatigue compared with controls, changes that were not evident in dystrophic soleus. Contrary to EDL fibers, soleus muscle fibers of Sgca-null mice distinctively showed right shift of the pCa-tension (pCa is the negative log of Ca2+ concentration) relationships and reduced sensitivity to caffeine of sarcoplasmic reticulum. Both EDL and soleus muscles showed striking changes in myosin heavy-chain (MHC) isoform composition, whereas EDL showed a larger number of hybrid fibers than soleus. In contrast to the EDL, soleus muscle of Sgca-null mice contained a higher number of regenerating fibers and thus higher levels of embryonic MHC. In conclusion, this study revealed profound distinctive biochemical and physiological modifications in fast- and slow-twitch muscles resulting from alpha-sarcoglycan deficiency (literal)
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