C L Defranco

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The present study relates duration of alcohol abuse and degree of cognitive impairment among a population of alcohol dependent subjects (DSM-III). Data on 175 consecutively admitted patients were first examined to empirically establish operational definitions of short-term and long-term alcohol abuse for this population. Five or more consecutive years of(More)
Incorporation of 32P from [gamma 32P]ATP into mannan could not be detected in homogenates of cultivated skin fibroblasts from patients with mucolipidosis II, and accounted for only up to 10% of normal control activity in cell lysates from patients with mucolipidosis III. Parents of patients with mucolipidosis II demonstrated 60-70% of normal control(More)
The activities of plasma and fibroblast cytidine 5'-monophosphate-sialic acid:glycoprotein sialyltransferases of patients with cystic fibrosis have been found to be within the range of activities of age- and sex-matched normal controls when asialofetuin served as the sialic acid acceptor. The use of desialylated preparations of purified human plasma alpha(More)
Increased incorporation of mannose into endogenous glycoprotein fractions has been found in whole cell lysates and crude membrane preparations of cultured skin fibroblasts from patients with cystic fibrosis (1.3-2.3-times normal) when GDP[14C]mannose served as the mannosyl donor. In contrast, the incorporation of mannose from GDPmannose into lipid fractions(More)
Purified preparations of plasma alpha 2-macroglobulin from patients with cystic fibrosis are shown to have normal amounts of total hexose but as much as 40% decrease in their sialic acid content. The binding of these preparations to concanavalin A and wheat-germ agglutinin was markedly reduced as compared to normal values in controls. Intermediate values(More)
The mechanism of E. coli chloramphenicol acetyltransferase (cat) mRNA decay was investigated. Alteration of the 5' untranslated terminus does not appear to have an effect on the turnover rate of the mRNA. Similarly, changes at the 3' terminus of the message, including the addition of a stable stem and loop structure, do not affect the half-life of the(More)
Fibroblasts from patients with mannosidosis, cultured in medium supplemented with fetal calf serum from which acidic alpha-mannosidase (alpha-D-mannoside mannohydrolase, E.C.3.2.1.24) has been removed, secreted a normal amount of apparently unaffected acidic alpha-mannosidase into fetal calf serum-free medium. Both the intracellular and extracellular acidic(More)
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