Wynik wyszukiwania w bazie Polska Bibliografia Lekarska GBL

Zapytanie: FERENS-SIECZKOWSKA
Liczba odnalezionych rekordów: 2



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Tytuł oryginału: Glycoforms of six serum glycoproteins in a patient with congenital disorder of glycosylation type I.
Autorzy: Ferens-Sieczkowska Mirosława, Zwierz Krzysztof, Midro Alina, Kątnik-Prastowska Iwona
Źródło: Arch. Immunol. Ther. Exp. 2002: 50 (1) s.67-73, il., tab., bibliogr. 29 poz.
Sygnatura GBL: 304,223

Hasła klasyfikacyjne GBL:
  • pediatria

    Typ dokumentu:
  • praca kazuistyczna
  • tytuł obcojęzyczny

    Wskaźnik treści:
  • ludzie
  • dzieci 13-18 r.ż.
  • płeć żeńska

    Streszczenie angielskie: In this paper the occurrence and relative content of defectively glycosylated serum glycoforms in transferrin (Tf), ŕ1-acid glycoprotein (AGP), haptoglobin (Hp), ŕ1-antitripsin (ŕ1-AT), ŕ2-macroglobulin (ŕ2-MG) and ceruloplasmin (Cpl) in the serum of a patient with congenital disorder of glycosylation type I are reported. Blood samples were taken when the patient was 14 years old and then after a one-year interval. The patterns of glycoforms in both samples were compared. In 4 out of 6 examined glycoproteins, glycoforms lacking one and two oligosaccharide chains occurred. "Underglycosylated" glycoforms of ŕ2-MG and Cpl were not clearly detectable. Tf was shown to be affected with this defect to a higher extent than other glycoproteins, containing only 30 p.c. properly glycosylated molecules and also as much as 30 p.c. of the molecules lacking two glycan units. In Hp and ŕ1-AT the proportions of properly and defectively glycosylated forms were similar. This properly glycosylated form comprised 47 p.c. of the Hp and 51-55 p.c. of the ŕ1-AT molecules. As in AGP and Tf, about 30 p.c. the of molecules lacked one glycan unit. Twenty-one percent of the Hp molecules were devoid of two glycans, and this amount slightly increased in the course of the year. In ŕ1-AT, 19 and 17 p.c. of the molecules lacked two glycans in both samples, respectively. Only in AGP we did find a substantial difference between the two blood samples. In the course of the year, the amount of the form lacking 2-chains decreases from 12 to 3 p.c., resulting in a simultaneous icrease in the forms lacking...

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    Tytuł oryginału: Skrypt do ćwiczeń z chemii medycznej dla studentów I roku wydziału lekarskiego i wydziału lekarsko-stomatologicznego : praca zbiorowa
    Autorzy: Ferens-Sieczkowska Mirosława, Gospodarek Aniela, Kątnik-Prastowska Iwona, Kłonowska Małgorzata, Kratz Ewa, Orczyk-Pawiłowicz Magdalena, Przybysz Magdalena, Pupek Małgorzata
    Źródło: - Wrocław, AM 2002, 120 s. : il., tab., 25 cm.
    Sygnatura GBL: 735,730

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