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Tytuł oryginału: Is the normal content of sulfhydryl groups attributable to sparing from dystrophic pathology in dystrophin-deficient muscles?
Autorzy: Niebrój-Dobosz Irena, Fidziańska Anna, Glinka Zofia, Hausmanowa-Petrusewicz Irena
Źródło: Folia Neuropathol. 2002: 40 (3) s.143-150, il., tab., bibliogr. 33 poz.
Sygnatura GBL: 304,961

Hasła klasyfikacyjne GBL:
  • pediatria
  • traumatologia i ortopedia

    Typ dokumentu:
  • praca kliniczna
  • praca doświadczalna
  • tytuł obcojęzyczny

    Wskaźnik treści:
  • ludzie
  • dzieci 6-12 r.ż.
  • dzieci 13-18 r.ż.
  • dorośli 19-44 r.ż.
  • zwierzęta
  • myszy

    Streszczenie angielskie: Deficiency of dystrophin in skeletal muscles is supposed to be responsible for all the symptoms associated with Duchenne dystrophy (DMD) and Becker dystrophy (BMD). The dystrophin-deficient mdx mice, however, are clinically almost asymptomatic. Hence, other factor(s) might be responsible for the muscle pathology in DMD and BDM. As sulfhydryl groups are involved in mainatining the structure of membranes and the protein-phospholipid interactions, total, protein-bound and free sulfhydryl groups (-SH) in DMD, BMD, limb-girdle dystrophy (LGMD) and the mdx mice muscles have been determined. A significant decrease of total and protein-bound - SH groups content and an increased proportion of free - SH groups in DMD and BMD was found. In LGMD the changes of total and protein-bound - SH groups content were less expressed. In the mdx mice muscles te content of - SH groups was generally normal, only a higher than normal proportion of free - SH groups content in old and senile animals, especially in their diaphragm, was present. To test the sercolemmal integrity, albumin/creatine kinase (CK) influx/efflux was determined. In early stages of DMD and BMD the albumin influx was increased. In advanced stages of these diseases albumin influx was not observed. In LGMD albumin penetration was present only in a few fibres. CK efflux in vitro was incrased both in early and advanced DMD cases. IN BMD adn LGMD CK efflux was increased only in early stages of the diseases. In mdx mice an increased influx/efflux of albumin/CK was stated in adult animals. The changes persisted in the mdx mice an increased influx/efflux...

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