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2 Archivum Immunologiae et Therapiae Experimentalis

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Glycoforms of six serum glycoproteins in a patient with congenital disorder of glycosylation type I

100%

Ferens-Sieczkowska M. , Zwierz K. , Midro A. , Katnik-Prastowska I.

Archivum Immunologiae et Therapiae Experimentalis

2002

tom 50

nr 1

67-74

In this paper the occurrence and relative content of defectively glycosylated serum glycoforms in transferrin (Tf), 1-acid glycoprotein (AGP), haptoglobin (Hp), 1-antitrypsin (1-AT), 2-macroglobulin (2-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 2-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% properly glycosylated molecules and also as much as 30% of the molecules lacking two glycan units. In Hp and 1-AT the proportions of properly and defectively glycosylated forms were similar. This properly glycosylated form comprised 47% of the Hp and 51?55% of the 1-AT molecules. As in AGP and Tf, about 30% 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 1-AT, 19 and 17% 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 decreased from 12 to 3%, resulting in a simultaneous increase in the forms lacking one chain and the properly glycosylated. Our work also indicates, that applying a simple method of biochemical analysis such as SDS-PAGE/Western-blotting could be helpful in preliminary diagnosis and could improve the identification of congenital disorders of glycosylation.

Time-dependent observations of secretion marker levels in nasal secretion after histamine and methacholine provocations

88%

Pupek M. , Mikulewicz W. , Mielnik J. , Batycka B. , Katnik-Prastowska I.

Archivum Immunologiae et Therapiae Experimentalis

2003

tom 51

nr 4

259-265

Nasal provocation tests with histamine and methacholine were carried out in 25 healthy men on an effort to assess the dynamic changes of albumin, total IgA, secretory IgA and lactoferrin concentrations in the nasal secretion. The trials were performed with 0.5, 1, and 4 mg of histamine and 8, 16, 32 mg of methacholine. Each dose of histamine or methacholine was sprayed into a nose every second day, and with two days interval between two provocating agents. The nasal secretions were collected after saline spraying only forming baseline group and after 3, 10 and 15 minutes of the challenge agent administration. The baseline levels presented the following values: for albumin 257? 230 mug/ml, secretory IgA 608 ? 379 mug/ml, total IgA 1025 ? 423 mug/ml, and lactoferrin 213 ? 156 mug/ml. The increase of albumin level after nasal provocation, particularly significant after histamine administration (to 3713 ? 2311 mug/ml), indicates the incessant protein plasma leaking from the blood circulation to the nasal secretion. After administration of both provocating agents there was the significant gradual decrease of secretory IgA level, even below the baseline value. After the 2nd and the 3rd doses of methacholine and histamine spraying the concentration of secretory IgA decreased 2-3 times and was found to be 200-300 mug/ml, respectively. Also, lactoferrin concentration values decreased gradually after the 2nd and 3rd doses of methacholine and histamine to level close the baseline value. These observations suggest time and dose dependent, a non-specific dysfunction of local immunity response after nasal provocations.