Enzymological studies on hereditary avian muscular dystrophy.

Penicillamine Treatment of Hereditary Avian Muscular Dystrophy

“…For the experimental determinations of titratable sulphydryl groups of soluble muscle proteins, the extracts from 5 normal and 5 dystrophic birds were assayed in the presence of 6 mol guanidine (Table 2). As we have previously shown (Patnode et al 1976), at the age of one year, dystrophic white muscle (pectoral) had only 18 % of the normal amount of SH groups per gram of muscle. On the basis of SH groups per mg of protein, dystrophic muscle had 53% of normal levels, indicating that there was a preferential loss of proteins with measurable sulphydryl Sulphydryl residues (SH) of proteins were determined in the presence of6 mol guanidine hydrochloride using the 5,5'-dithiobis (2-nitrobenzoic acid) method (Ellman 1959).…”

“…These determinations demonstrate the extent of the deficiencies in sulphydryl proteins in dystrophic muscles. In recent preliminary studies, we have shown that penicillamine treatment raises the SH level of dystrophic muscle proteins to normal values (Hill et al 1976).…”

“…Our recent studies with penicillamine treatment indicate that the dehydrogenase activity in white dystrophic muscle is retained but not completely restored to normal values (Hill et al 1976). The specific activity of treated birds at four months was preserved at about 75 % of normal activity values.…”

Η Ακυλο Φωσφαταση Απο Ερυθροκυτταρα Ενηλικος Και Ομφαλιου Λωρου

“…( Προσδιορίσθηκε ή δραστικότητα αύτων των δύο ένζυμων άπό τίς κόκκινες καί άσπρες μυϊκές ίνες κότας πού πάσχει άπό μυϊκή δυστροφία καί έγινε σύγκριση μέ τίς τιμές της δραστικότητας τοϋ ένζυμου, άπό τίς φυσιολογικές μυϊκές ίνες κότας (105). Άπό τά ευρήματα αυτής της εργασίας, συγκεντρώ νονται αρκετές ενδείξεις, γιά τή συμμετοχή των σουλφυδρυλομάδων στή παθογένεση της μυϊκής δυστροφίας.…”