Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “GALACTOSE”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 1,495 records · Page 83Linked to original sources

Galactose neuropathy. Permeability studies, mechanism of edema, and mast cell abnormalities.

Galactose neuropathy is characterized by progressive endoneurial edema manifested by a gradual increase in endoneurial fluid pressure. Edema accumulates via a unique mechanism of osmotic force generated by products of the polyol pathway, synthesized within the endoneurial compartment. This paper presents morphologic findings showing firstly, that blood nerve barrier permeability to horseradish peroxidase complexes appears unchanged and secondly, peripheral nerve edema in this condition is restricted to extraganglionic endoneurium sparing the spinal ganglia and adjacent roots. Thirdly, mast cells accumulated in significant numbers and electron microscopy revealed degranulation. There was no evidence of edema in Schwann cell cytoplasm, the putative site of galactitol accumulation via the sorbitol pathway. These findings are discussed with respect to diabetic neuropathy for which galactose intoxication is a useful experimental model.

Animals↗

Versatile synthesis of bi- and tri-antennary galactose ligands: interaction with the Gal/GalNAc receptor of human hepatoma cells.

We have synthesized bi- and tri-antennary galactose ligands by coupling 1-thio-beta-D-galactose derivatives to the alpha- and epsilon-amino groups of L-lysine and L-lysyl-L-lysine via highly flexible hydrophilic spacer arms that allow variation of their intergalactose distances. The interaction of these ligands with the Gal/GalNAc receptor of HepG2 cells showed a binding affinity that was: (i) in agreement with the clustering effect known to occur with more complex oligomeric structures, i.e. tri- > bi-antennary; ii) dependent on the intergalactose distances (optimal interactions were observed for the tri-antennary structures with distances > 2 nm). These ligands, that can be easily conjugated to bioactive (macro) molecule carrier systems, could be useful for their targeting to hepatocytes.

Animals↗

Characterization of novel mono-O-acetylated GM3s containing 9-O-acetyl sialic acid and 6-O-acetyl galactose in equine erythrocytes.

Novel mono-O-acetylated GM3s, one containing 9-O-acetyl N-glycolyl neuraminic acid and another containing 6'-O-acetyl galactose, were isolated as a mixture from equine erythrocytes, and the structures were characterized by one- and two-dimensional proton nuclear magnetic resonance (NMR) and fast atom bombardment-mass spectrometry (FAB-MS). The position of the O-acetyl residue was identified by the downfield shift of the methylene protons at C-9 of N-glycolyl neuraminic acid (9-O-Ac GM3) and C-6 of galactose (6'-O-Ac GM3) in the NMR spectrum, in comparison to the respective non-acetylated counterparts. To confirm the presence of 6'-O-Ac GM3, the O-acetylated GM3 mixture was desialylated with Arthrobacter neuraminidase, giving 6-O-acetyl galactosyl glucosylceramide, the structure of which was estimated by NMR and FAB-MS, together with non-acetylated lactosylceramide with a ratio of 1:1.

Acetylation↗

Ultrastructural visualization of galactose in the glycoprotein of gastric surface cells with a peanut lectin conjugate.

The ultrastructural localization of peanut lectin-binding sites in the gastric surface epithelial cell has been studied using a horseradish peroxidase-labelled peanut lectin (PL-HRP) conjugate and other cytochemical techniques. The PL-HRP procedure has visualized glycoprotein with presumed terminal galactose residues in the apical plasmalemma and secreted mucins and has localized such glycoprotein selectively in the 'intermediate Golgi cisternae' situated between the saccules of the maturing face and those of the forming face of the Golgi stacks. Other cytoplasmic organelles, including the forming and stored secretory granules, did not reveal glycoprotein with terminal galactosyl residues. These results demonstrate the applicability of the PL-HRP labelling technique at the electron microscopical level to localize not only extra but also intracellular peanut lectin-reactive sites. The observation afford information concerning the possible site in the Golgi apparatus where galactose residues are added to the growing oligosaccharide side-chain of mucous glycoprotein.

Animals↗

Growth rate, muscle protein composition, intestinal absorption of D-galactose and L-leucine and nitrogen balance in rats fed the raw legume Chamaecytisus proliferus L.

Growth rate, nitrogen balance, skeletal muscle nitrogen fractions and in vivo intestinal absorption of D-galactose (2 mM) and L-leucine (20 mM) have been measured in male growing rats (90-100 g initial body weight) fed 12% protein diets containing either casein (control) or the raw leafy legume Chamaecytisus proliferus L. (Western Canary Islands). As compared to control casein-fed rats, those fed the raw legume diet exhibited a significant reduction (P less than 0.01) in the rate of growth, nitrogen balance parameters, sarcoplasmic nitrogenous fraction of skeletal musculature and in vivo intestinal absorption of galactose and leucine; however, the non-protein nitrogenous fraction has been found significantly higher (P less than 0.01) than that of control animals, whereas the myofibrillar nitrogenous fraction remained unchanged. The possible nature of these findings is discussed.

Animals↗

The UDP-galactose translocator gene is mapped to band Xp11.23-p11.22 containing the Wiskott-Aldrich syndrome locus.

We have cloned a segment of the human gene encoding UDP-galactose translocator by genetic complementation of its defective mutant in mouse FM3A cells. Chromosome mapping using fluorescent in situ hybridization revealed that the cloned gene hybridized to the Xp11.23-11.23 region of the X chromosome. This region is shared by the locus of Wiskott-Aldrich syndrome, an X-linked recessive immunodeficiency disorder, characterized by defective sugar chains on cell surface components. Genetic and phenotypic similarities suggest a possible link between UDP-galactose translocator and the Wiskott-Aldrich syndrome (WAS).

Animals↗

Increased serum urate in galactosemia patients after a galactose load: a possible role of nucleotide deficiency in galactosemic liver injury.

Five galactosemic and 5 normal children received an oral load of galactose under standardized conditions. The maximal blood galactose level after 1.5 hours was 12.6 +/- 2.0 (S.D.) mmol/l in individuals with a deficiency of uridylyl transferase (EC2.7.7.12) as compared to 5.8 +/- 1.2 (S.D.) mmol/l in the controls. The concentration of serum urate in galactosemics increased to 155% of the fasting level (P less than 0.005); no rise was detectable in the controls. The elimination of urate with the urine was augmented by the same amount in both groups. Our studies provide evidence for an increased catabolism of hepatic nucleotides. This may lead to a deficiency of nucleotides which is proposed as a cause of galactosemic liver injury.

Blood Glucose↗

Fruits and vegetables are a source of galactose: implications in planning the diets of patients with galactosaemia.

It has become apparent that removing dairy products from the diets of patients with galactosaemia does not sufficiently diminish the deleterious signs. We have determined the amount of soluble monomeric galactose in 45 fruits and vegetables using capillary gas chromatography and selective ion monitoring. Galactose contents ranged from less than 0.1 mg per 100 g of tissue in artichoke, mushroom, olive, and peanut to 35.4 mg per 100 g in persimmon. Fruits and vegetables with over 10 mg per 100 g included date, papaya, bell pepper, tomato and watermelon. These results will provide important data for planning the diets of patients with galactosaemia.

Fruit↗

Prevention of experimental liver metastases by D-galactose.

The metastasis of malignant tumors from a primary site to near and distant secondary sites is probably the most important event in the pathogenesis of cancer and it accounts for most cancer deaths. Whereas advances in the treatment of primary cancer have led to increased patient survival, metastatic cancers are still the most difficult group of diseases to treat successfully. As organ-characteristic lectins play an important role in the organ manifestation of metastatic islets, it might be possible (e.g. during surgical operations on malignant tumors) to block those organ-characteristic lectins with the appropriate receptor-bearing glycoconjugates in order to inhibit the metastatic spread. Recent experiments have demonstrated that neuraminidase treatment of tumor cells (mouse sarcoma-1) alters in vivo (Balb/c-mice) the organotropic distribution of metastases; instead of being found exclusively in the lung, they are found both in lung and liver. However, pre-injection and regular application of D-galactose--the same holds for arabinogalactan--prevents the setting of metastases in the liver but does not influence the metastatic process to the lung, whereas mannan--as a galactose-free control substance--does not alter the initial pattern of metastasis to lung and liver.

Animals↗

Analysis of the relationship between bacterial adherence and extracellular production of mannose, galactose, glucose and ribose in Staphylococcus epidermidis and Staphylococcus hominis.

Gas-liquid chromatography-mass spectrometry was used to analyze the extracellular extracts of 108 coagulase-negative staphylococcal strains for the presence of mannose, galactose, glucose and ribose, in order to determine whether production of these four monosaccharides, regarded as potential staphylococcal slime components, was associated with the adherence capacity of the individuals strains. A total of 90 Staphylococcus epidermidis and 18 Staphylococcus hominis strains were studied. Using the quantitative spectrophotometric assay, 21 Staphylococcus epidermidis strains were classified as strongly adherent, 12 as moderately adherent, 11 as weakly adherent, and 46 as nonadherent. All 18 Staphylococcus hominis strains were nonadherent. Mannose, galactose, glucose and ribose were detected as the main monosaccharide components in the extracellular extracts of all strains examined. Moreover, the mean relative concentrations of these monosaccharides were essentially the same for the different adherence phenotypes within the species Staphylococcus epidermidis. These results showed that there was no causal connection between the adherence of coagulase-negative staphylococci and the extracellular production of any of the four monosaccharides analyzed.

Bacterial Adhesion↗

Galactose and leucine transport in the developing rat small intestine.

Jejunal transport of galactose and leucine was studied in 9--45 day-old rats by means of the everted sac technique. Maximum transport was observed in 9--10-day-old rats, but then decreased until the 22nd day, and remained unchanged from then on. There were no remarkable differences in the pattern of galactose and leucine transport over this period.

Animals↗

Glycogen storage disease, Fanconi nephropathy, abnormal galactose metabolism and mitochondrial myopathy.

We present a 4-year-old male suffering from profound muscular weakness, enzymatically undefined glycogen storage disease. Fanconi nephropathy and impaired galactose utilization. Distorted mitochondria, intramitochondrial fat droplets and partial deficiencies of pyruvate dehydrogenase complex, succinate: cytochrome c oxidoreductase, and cytochrome c oxidase have been found in muscle tissue. The causal relationship between mitochondrial myopathy, glycogen storage disease, Fanconi nephropathy and impaired utilization of galactose is discussed.

Child, Preschool↗

Effects of in situ and systemic lindane treatment on in vivo absorption of galactose and leucine in rat jejunum.

In vivo intestinal absorption of L-leucine is significantly decreased by the presence of lindane (0.3, 0.2 and 0.1 mM) in perfusion solution (in situ lindane treatment) for 5 min. The inhibitory effect is earlier when lindane concentration is higher, and it is irreversible. There are no changes in D-galactose absorption when lindane (0.3 and 0.2 mM) is perfused for 5 min, but a significant decrease in observed if pesticide is perfused for 10 min at 0.3 mM concentration. Subcutaneous lindane treatment at a dose of 68.76 mumol/kg over 7 days does not alter D-galactose and L-leucine absorption. In situ lindane treatment (0.3, 0.2 and 0.1 mM) induces a significant decrease in basolateral (Na(+)-K(+)-ATPase activity. In contrast, systemic lindane treatment (s.c. injection) at doses of 34.38 and 68.76 mumol/kg over 7 days does not alter this enzyme activity, although a significant decrease is observed in rats injected s.c. with 68.76 mumol/kg lindane over 15 days.

Animals↗

The beta-galactosidase activity in Kluyveromyces marxianus CBS6556 decreases by high concentrations of galactose.

In this paper we report on the effect of different concentrations of lactose and galactose in the production of beta-galactosidase by Kluyveromyces marxianus CBS6556. The results clearly demonstrate a decrease in enzyme specific activity during cultivation at high concentrations of L-lactose or D-galactose, despite the fact that these carbohydrates are normally used for induction of the beta-galactosidase activity. Therefore, maximum induction of beta-galactosidase in K. marxianus batch cultures was obtained at low concentrations of the inducer carbohydrates, in the range between 0.5 to 15 mM. Those informations can help to design low cost medium with higher beta-galactosidase productivity by K. marxianus cells.

Culture Media↗

Inhibitory effect of orally administered aldose reductase inhibitor SNK-860 on corneal polyol accumulation in galactose-fed rats.

BACKGROUND: Diabetic keratopathy, frequently observed after vitreous surgery, has been thought to be related to the aldose reductase-catalyzed reaction. However, few reports have been published on the chronological changes in polyol accumulation and the effect of the aldose reductase inhibitor in the corneal epithelium or endothelium of galactosemic rats. Consequently, the polyol accumulation in corneal epithelium and endothelium with stroma of 50% galactose-fed rats and the preventive effect of an aldose reductase inhibitor, SNK-860, were biochemically analyzed. METHODS: Four-week-old male Sprague-Dawley rats were fed a 50% galactose diet without supplement or supplemented with a low (3 mg/kg b.w.) or high (30 mg/kg b.w.) dose of SNK-860 (Sanwa Kagaku Kenkyusho, Japan), or a normal diet. Polyol contents in the corneal epithelium or endothelium with stroma were individually measured using gas-liquid chromatography. RESULTS: Polyol in corneal epithelia accumulated quickly in the 1st week, reached a maximum at the 3rd week of feeding and then gradually decreased. Low- and high-dose SNK-860 treatment significantly inhibited polyol accumulation in the epithelium and endothelium with stroma, respectively. Changing to the normal or SNK-860 supplemented diets significantly inhibited polyol accumulation. CONCLUSION: This finding indicates that oral administration of a new aldose reductase inhibitor, SNK-860, or systematic treatment of diabetes may be effective in preventing polyol pathway-induced corneal damage by quickly reducing the polyol level.

Administration, Oral↗

Dbf2 is implicated in a Cbt1-dependent pathway following a shift from glucose to galactose or non-fermentable carbon sources in Saccharomyces cerevisiae.

Dbf2, a cell cycle-regulated protein kinase, has been shown recently to be part of the CCR4 transcriptional regulatory complex in the yeast Saccharomyces cerevisiae. We report here that temperature-sensitive (ts) dhf2-2 mutant cells can be rescued by overexpression of CDC14, which encodes a dual-specificity protein phosphatase, when grown on glucose-containing medium, as reported previously, but not on galactose. Screening of two S. cerevisiae cDNA libraries led to the identification of CBT1 as a gene which, if overexpressed simultaneously with CDC14, results in the rescue of the dbJ2-2 mutation at restrictive temperature on galactose-based medium, as well as on media containing non-fermentable carbon sources such as glycerol, lactate and acetate. Cbt1 has been shown previously to be essential for formation of cytochrome b in the mitochondria. On the other hand, the ts defects of ccr4delta and caf1delta mutants on glycerol medium at 37 degrees C (Ccr4 and Caf1/Pop2 are two other members of the CCR4 complex) could not be complemented by simultaneous overexpression of CBT1 and CDC14. CCR4 and CAF1 have been shown to play an essential role in activating the expression of genes for non-fermentative growth. Our results strongly suggest that, within the CCR4 complex, Dbf2 is directly involved in some mitochondrial function that depends on cytochrome b or on one of its main regulators, Cbt1. Therefore, Dbf2 may be required not only during mitosis but also during growth on non-fermentable carbon sources.

Carbon↗

Nephrolithiasis in a child with glucose-galactose malabsorption.

Glucose-galactose malabsorption (GGM) is a rare autosomal recessive disorder of intestinal transport of glucose and galactose, leading to watery diarrhea, dehydration, failure to thrive, or early death. We report a female newborn with GGM, whose clinical diagnosis was confirmed by mutational analysis of the SGTL1 gene. Bilateral nephrolithiasis was discovered after an episode of hematuria. Metabolic causes of nephrolithiasis were not found. The most likely explanation for the development of nephrolithiasis is chronic diarrhea leading to dehydration and highly concentrated urine. High fluid intake and rigorous prevention of dehydration is therefore advised for these patients. Furthermore, life-long monitoring of their renal status, including regular ultrasound examinations, is warranted.

Amino Acid Transport Systems, Basic↗