Galactose deficiency

Recent studies on the biosynthesis of part of the carbohydrate portion of LPS have indicated that lipid can play a key role in this process in at least two ways. A study of the enzymatic transfer of glucose and galactose (as sugar nucleotides) to glucose- or galactose-deficient LPS of Salmonella mutants has revealed a specific requirement for lipids (Rothfield and Horecker, 1964). The most effeetive lipid is phospha-... 

Glucose/galactose malabsotption (GGM) is an intestinal monosaccharide (glucose and galactose) transport deficiency. The disorder manifests itself within the first weeks of life. The severe diarrhea and dehydration are usually fatal unless glucose and galactose are eliminated from the diet. Fiuctose and xylose are absorbed normally. Occurrence in both males and females, familial incidence, in particular in parental consanguinity, indicate autosomal recessive inheritance of... 

L The answer is a. (Hardman, p 922) Lactulose is a synthetic disaccharide (galactose-fructose) that is not absorbed. In moderate doses, it acts as a laxative. In higher doses, it is capable of binding ammonia and other toxins that form in the intestine in severe liver deficiency and that are believed to cause the encephalopathy. Loperamide is an antidiarrheal opioid lorazepam is a CNS depressant loxapine is a heterocyclic antipsychotic. 

Deficiency of galactose 1-phosphate uridyltransferase produces a more severe disease because, in addition to galactosemia, galactose 1-P accumulates in the liver, brain, and other tissues. The two enzyme deficiencies are compared in Table 1-12-3. 

Galactokinase Deficiency Galactose 1-Phosphate Uridyltransferase Deflcieucy... 

In a person with glucose 6-phosphatase deficiency, ingestion of galactose or fructose causes no increase in blood glucose, nor does administration of glucagon or epinephrine. 

Galactose 1-phosphate uridyl transferase deficiency (galactosemia)... 

Galactose intolerance Voriconazole tablets contain lactose and should not be given to patients with rare hereditary problems of galactose intolerance, Lapp lactase deficiency, or glucose-galactose malabsorption. 

Classic galactosemia is a rare, autosomal recessive disorder caused by deficiency of galactose 1-phosphate uridyltransferase. 

Fermentation procedures useful for the production of uridine 5 -(a-D-galactopyranosyl pyrophosphate) involve the cultivation of bacterial mutant-strains that are deficient in the 4"-epimerase for 30 (see Section V,l,b, p. 369) in D-galactose-containing media,245-247 or by incubating Torulopsis Candida cells with uridine 5 -phosphate, D-galactose, potassium phosphate, and magnesium sulfate.248... 

A.l. 4 Enzyme Assay for CDG-lld (Golgi UDP-Galactose N-Acetylglucosamine /3-1,4-Galactosyltransferase I Deficiency [11])... 

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