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PubMedDate not supplied

Effects of cataractogenesis on the CDP-choline pathway: increased phospholipid synthesis in lenses from galactosemic rats and 13/N guinea pigs.

We investigated the effects of cataractogenesis on phospholipid (P-lipid) synthesis in sugar cataracts from galactosemic rats and in hereditary cataracts from 13/N guinea pigs. Cataractous lenses from rats fed a 50% galactose diet for 7 days were incubated 24 h with radiolabeled choline or ethanolamine and the P-lipids were extracted. The galactosemic cataracts synthesized twice as much phosphatidylcholine (PtdCho) as control rat lenses, and phosphatidylethanolamine synthesis also was increased. Similar analysis of cataractous lenses from 3-week-old 13/N guinea pigs showed a 3-fold increase in PtdCho synthesis compared with control lenses. In all cases, the P-lipid precursor pool was lower in cataracts than in control lenses. The increased P-lipid synthesis in these cataracts may represent a membrane repair response to cataractogenic stress.

Animals↗
PubMed1993

The interval between the positive peak of premyoclonus spike and the onset of myoclonus is shorter than the cortical latency in cortical myoclonus.

In 6 patients with cortical myoclonus, the intervals between the positive peak of the premyoclonus cortical spike and the onset of the myoclonic jerk [interval (Sp-Mcl)] were precisely measured by the computer-assisted jerk-locked averaging method. The intervals (Sp-Mcl) were 13.0-15.8 ms for the abductor pollicis brevis, 8.0-10.4 ms for the extensor digitorum communis and 5.5 ms for the biceps brachii, respectively. These were apparently shorter than the normal latencies of the responses elicited by transcranial cortical stimulation. The difference between the interval (Sp-Mcl) and the spinal latency was -0.8 to 3.5 ms, which was not long enough for the time required for the descending volleys going down from the cortex to the spinal cord. We have concluded that the motor cortex is activated before the positive peak of the premyoclonus spike in cortical myoclonus.

Adult↗
PubMed1991

Postprandial blood concentrations of insulin-independent carbohydrate, galactose, in oral test after gastric surgery.

In order to examine the postprandial blood concentrations of insulin-independent carbohydrates after gastric surgery oral galactose test (1.65 g/kg body weight in water, 33%, w/v) was performed in 55 symptomatic patients and in 5 healthy subjects. There were patients after total gastrectomy (TG) (n = 17), gastric resection with (GRS, n = 17) or without (GR; n = 12) selective vagotomy, and after proximal selective vagotomy (PSV, n = 9). The patients had immediately after drinking the test solution a 2- to 5-fold higher blood galactose concentration than the healthy subjects. The TG patients had the most rapid, the healthy subjects the slowest and the GR and GRS patients an intermediate rapid, immediate increase of blood galactose concentration. The TG patients showed a plateau 40-60 min and a decrease 60-90 min after the start of the test. The PSV patients showed a plateau 60-90 min after the commencement of the test. The GR and GRS patients and the healthy subjects had a continuous increase in blood galactose concentration during the whole test period, but the maximal point 90 min after the drinking of the solution was lower in the GRS than in the GR patients and lowest in the healthy subjects. The PSV patients had a lower blood galactose curve than the TG, GRS and GR patients but higher than the healthy subjects except the plateau 60-90 min postprandially.(ABSTRACT TRUNCATED AT 250 WORDS)

Carbohydrate Metabolism↗
PubMed1997

Blood clearance of 99mTc-trimethyl-Br-IDA discriminates between different degrees of severe liver ischaemia--reperfusion injury in the rat.

The kinetics of 99mTc-Trimethyl-Br-IDA blood clearance was analysed in the rat 24 h after warm ischaemia and reperfusion of the liver. There were changes in the elimination of 99mTc-Trimethyl-Br-IDA depending on the length of the ischaemic period and the dose given. Statistically significant differences were found between the various periods of ischaemia when higher doses of the radionuclide were utilised. At lower doses, the clearance was not capable to discriminate between control rats and rats submitted to 45 min of ischaemia, but it did discriminate more severe degrees of ischaemic liver injury. Instead, galactose elimination capacity discriminated between ischaemic and control rats, but not between 45 and 90 min or between 90 and 120 min of ischaemia. Alanine aminotransferase was able to discriminate between control and ischaemic rats and between 45 and 90 min of ischaemia, but not between 90 and 120 min of ischaemia. The response of 99mTc-Trimethyl-Br-IDA clearance under extreme conditions of ischaemia and reperfusion is consistent and opens a possible window for the application of this test in the quantification of liver function in severely damaged livers and in decision making and prognosis in liver disease.

Alanine Transaminase↗
PubMed1982

Polymorphism of human galactose-1-phosphate uridyl transferase.

Frequencies for the electrophoretically detectable alleles of galactose-1-phosphate uridyl transferase were determined from 406 neonates in Adelaide. GALT*D and GALT*LA frequencies were 0.054 and 0.027, respectively. The expected proportion of heterozygotes was 15.3%, with a GALT N-D component of 10%. The distinction between GALT*D and GALT*LA is essential for estimation of the expected frequency of GALT DG genotypes, an unknown proportion of which are detected as 'deficient' from neonates on galactosaemia screen.

Alleles↗
PubMed1984

Polymorphism of erythrocyte galactose-1-phosphate uridyl-transferase in Italy: segregation analysis in 693 families.

In the course of a population study in Italy, blood samples collected from 802 unrelated newborns and both their parents (when possible) have been examined for galactose-1-phosphate uridyltransferase (GALT) polymorphism. Electrophoresis and quantitative assay of GALT activity were not always sufficient for an accurate identification of the different GALT genotypes; segregation analysis provided better criteria for classification. A parent-child correlation coefficient for GALT activity equal to 0.107-0.155 was found when only the transmission of the normal allele was concerned, but the correlation rose to 0.618-0.682 when the Duarte and Los Angeles alleles were segregating. This confirmed the existence of a low (Duarte) and high (Los Angeles) activity variant. The overall validity of our genotype classification is supported by the good agreement between observed and expected mating types and segregations. The following gene frequencies were found for the different alleles: N = 0.9192, G = 0.0036, D = 0.0372 and LA = 0.0400.

Adult↗
PubMed1982

Metabolic effects produced in baboons associated with the ingestion of diets based on lactose hydrolysate.

Diets containing 80% by weight carbohydrate provided by lactose hydrolysate (prepared by two different techniques), sucrose or glucose were given to baboons for 10-week periods. Fasting blood samples were taken regularly and the serum was assayed for galactose, glucose, insulin, triglyceride and cholesterol. The body weight of the animals was recorded. During both lactose hydrolysate dietary periods the mean fasting serum galactose concentration increased whereas the fasting serum glucose level fell initially. The fasting serum glucose concentration rose with both the glucose and the sucrose diets. The fasting serum triglyceride concentration of the female baboons increased significantly during both periods of ingestion of lactose-hydrolysate-based diets whereas the glucose and sucrose diets did not produce a significant effect. The sucrose diet produced a consistent increase in the mean triglyceride level of the male baboons. The mean serum cholesterol fell in the male and female baboons during all four diets.

Animals↗
PubMed1989

Effect of 5% galactose diet on galactose and dulcitol in plasma and lens of male and female pigs.

The study was performed to compare the galactose tolerance of male and female pigs to 5% galactose and 25% hydrolysed whey (HW; containing 5% galactose) diets. Plasma galactose and dulcitol levels were measured throughout the diet period and correlated with the lens dulcitol contents at the end of the period. On the first day of the diet there was no significant difference between male and female blood galactose levels after galactose ingestion. The consumption of HW resulted in a significant 30% decrease in the blood galactose peak in females; there was no parallel difference in males on the HW diet. The 30-day diet induced a significant decrease in galactosaemia peaks resulting from galactose ingestion in both males and females, indicating an activation of galactose metabolism. The HW diet resulted in an equivalent decrease in peak galactosaemia only in males, cancelling the initial differences observed between males and females. The final dulcitol content in the lenses appeared to be correlated with plasma galactose but not with plasma dulcitol. However, the differences in plasma galactose between males and females, or between the two diets, resulted in smaller and often non-significant differences in lens dulcitol.

Analysis of Variance↗
PubMed1990

Effect of prolonged galactose consumption on galactose tolerance in young healthy humans.

This study was carried out on two groups of healthy young subjects. One group (n = 8) was given a single oral dose of galactose (0.5 g/kg body weight), and a single measurement of blood galactose was made 40 min later. A second group (n = 16) was given a daily supplement of 0.5 g/kg galactose for 15 days, and blood galactose and galactitol were measured. On the first day of the test period, some subjects seemed to tolerate galactose less well than others: galactitol appeared in their plasmas and could be related to a significantly higher galactosaemia than that of subjects without detectable galactitol in the plasma. However, after 15 days of galactose intake, the less galactose-tolerant subjects appeared to have adapted, as indicated by the significant decrease in the blood galactose and the disappearance of galactitol. No change was found in the galactose-tolerant subjects. No significant difference was found between males and females.

Adult↗
PubMed1990

Kinetics and balance of glucose and galactose appearance in the portal blood after intake of lactose or hydrolysed lactose in conscious pigs.

Five pigs (mean body weight: 66.2 kg) were fitted with portal and arterial catheters and an electromagnetic flow probe around the portal vein. One week after the surgical operation, each animal was successively fed, at 3-day intervals, with two experimental meals containing 392 g dry matter either from hydrolysed or non-hydrolysed lactose together with a protein-mineral-vitamin mixture (200 g). Portal and arterial blood concentrations of glucose, galactose and amino nitrogen were measured together with the portal blood flow rate during a postprandial period of 8 h after the intake of these experimental meals. Amounts of hexoses appearing in the portal blood after hydrolysed-lactose intake were 3- and 2-fold larger within 2 and 8 h, respectively, than after non-hydrolysed-lactose intake. Thus, enzymatic hydrolysis in the intestine is the limiting factor of lactose digestion in unadapted pigs. Whatever the type of lactose ingested, glucose appeared more rapidly and in larger amounts in the portal blood than galactose. After hydrolysed-lactose intake, the amount of glucose appearing in the portal blood exceeded the amount ingested already after 5 h. This means that a fraction of galactose was transformed into glucose during the transport by the enterocyte.

Animals↗
PubMed1975

Concentration and transport of different sugars in the lymph of the thoracic duct and in blood of the portal vein in dogs after enteral and parenteral administration.

The uptake and distribution of glucose, galactose, fructose and inulin in dogs was investigated in the lymph of the thoracic duct and in different blood vessels after enteral and parenteral administration. Whereas inulin could be detected neither in the lymph nor in portal venous blood after enteral administration, all other sugars were found in different concentrations in blood and lymph. Although the concentration of different sugars in the lymph after enteral and parenteral application can be compared to that in serum, the amount of sugars transported via the lymphatics is so small that it can be neglected.

Animals↗
PubMed1988

Patterns of serum glucose and galactose concentrations in term newborn infants after milk feeding.

Galactose and glucose concentrations were measured in peripheral blood in relation to ad libitum milk feeding in 11 healthy near-term infants. Galactose and glucose concentrations before feeding averaged 1.06 +/- 0.21 and 60.3 +/- 3.2 mg/dl, respectively. After feedings containing 0.76-2.68 g lactose/kg body weight, both galactose and glucose rose by approximately 35% Galactose concentrations rose by 0.72 +/- 0.10 mg/dl at 30 min after feeding, while glucose concentration rose by 23.3 +/- 2.5 mg/dl at 30 min after feeding. The 25-fold greater absolute increase in the concentration of glucose than galactose is consistent with efficient first-pass clearance of galactose by the neonatal liver.

Analysis of Variance↗
PubMed1972

The aminoacidopathies.

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Amino Acid Metabolism, Inborn Errors↗