Identification of two genetic variants of goat alpha-lactalbumin.
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Biomedical subjects
Publications and source records attributed to E Maes.
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beta(1-4)-Galactosyltransferase from human milk (the A protein of lactose synthase) has been found to be heterogeneous when fractionated by affinity chromatography against insolubilized alpha-lactalbumin, using a linear gradient of decreasing N-acetylglucosamine concentration. Three forms were isolated. Molecular weights of the different species, as determined by sodium dodecylsulphate gel electrophoresis, were found to be 38 000, 43 000 and 50 000. The 38 000 and 50 000 species were studied for their catalytic ability to synthesize either lactose in the presence of alpha-lactalbumin, or N-acetyllactosamine in the presence and absence of the 'specifier' protein. Appreciable difference was observed between the two enzyme forms with respect to their catalysis of lactose synthesis with alpha-lactalbumins from various sources. Differences in the rate of production of N-acetyllactosamine in the presence of alpha-lactalbumin were also observed. For the lowest-molecular-weight species it was found that the inhibitory effect of alpha-lactalbumin upon N-acetyllactosamine synthesis becomes an activating effect at higher alpha-lactalbumin concentrations, while no such inversion was observed for the other species. The results suggest that the conformation at the site of association of the enzyme with the acceptor saccharide or alpha-lactalbumin has been changed, presumably by a pratial enzymic hydrolysis.
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A 50 year old man is described in whom primary hypothyroidism and isolated ACTH deficiency leading to adrenocortical insufficiency occurred simultaneously. Low thyroid hormone levels, elevated serum TSH values and high titers of antithyroid antibodies (ATA) were consistent with primary hypothyroidism. The diagnosis of secondary adrenocortical insufficiency was based on low cortisol and ACTH morning levels, low urinary steroids, a significant increase of cortisol levels after 0.25 mg tetracosactide iv, an absence of ACTH and cortisol release after insulin-induced hypoglycemia and a similar absence of ACTH and 11-deoxycortisol increase after metyrapone administration. After one year treatment and five days withdrawal of cortisol administration, the insulin-induced hypoglycemia still failed to elicit secretory responses of ACTH and cortisol, despite the normalization of thyroid hormone levels. The etiopathogenesis of these two coexisting endocrine deficiencies is discussed.
Lyme disease produces a diverse clinical picture that can include serious and potentially debilitating cardiac, neurologic, joint, and skin involvement. It is characterized in three stages--early localized (stage I), early disseminated (stage II), and late disseminated (stage III)--and medical management is highly dependent on the stage at which the patient presents and the physician's awareness of available treatment options. This study was conducted to establish the medical and economic burden of Lyme disease in the overall US population, which included determining its endemicity in high-risk states and counties, describing current treatment patterns, measuring direct and indirect costs, and defining the cost burden by age group (<18 years and > or =18 years of age). Medical, epidemiologic, and economic data were collected, and an algorithm was developed representing the natural course of Lyme disease and the progress of health states over time following medical intervention. Using an annual mean incidence of 4.73 cases of Lyme disease per 100,000 population in the decision analysis model yielded an expected national expenditure of $2.5 billion (1996 dollars) over 5 years for therapeutic interventions to prevent 55,626 cases of Lyme disease sequelae. This estimate included both direct medical and indirect costs. However, there is evidence of considerable variation in incidence within states. Our findings support development of vaccination strategies for specific target groups.
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Presentation of a case of Sipple's syndrome with bilateral malignant pheochromocytoma, bilateral thyroid medullary carcinoma and two parathyroid adenomas. The principal characters of the syndrome, the diagnostic tests, the necessity of a prospective survey of family relatives are discussed.