Page last updated: 2024-09-03

galactose-1-phosphate and galactose

galactose-1-phosphate has been researched along with galactose in 76 studies

Compound Research Comparison

Studies
(galactose-1-phosphate)
Trials
(galactose-1-phosphate)
Recent Studies (post-2010)
(galactose-1-phosphate)
Studies
(galactose)
Trials
(galactose)
Recent Studies (post-2010) (galactose)
17023718,9881934,605

Research

Studies (76)

TimeframeStudies, this research(%)All Research%
pre-199021 (27.63)18.7374
1990's8 (10.53)18.2507
2000's30 (39.47)29.6817
2010's12 (15.79)24.3611
2020's5 (6.58)2.80

Authors

AuthorsStudies
Bozkowa, K; Cabalska, B; Grodzka, Z; Zbieg-Sendecka, E1
Angus, PW; Collins, JE; Dixon, RM; Kalderon, B; Leonard, JV; Oberhaensli, RD; Radda, GK; Rajagopalan, B1
Lemonnier, A; Mangeot, M; Pourci, ML; Soni, T1
Hata, M; Ichiba, Y; Inoue, B; Misumi, H; Mori, T; Wada, H1
Hartmann, PE; Riordan, PR; Wilson, RG1
Dilling, LA; Ford, JD; Greenberg, CR; Haworth, JC; Seargeant, LE; Thompson, R1
Cherruau, B; Djabal, A; Lemonnier, A; Mangeot, M; Myara, I; Pourci, ML1
Gałasiński, W; Kopacz-Jodczyk, T1
Guthrie, R; Jinks, DC; Orfanos, AP1
Barth, CA; Kopra, N1
Chassy, BM; Thompson, J1
Henderson, MJ; Holton, JB; MacFaul, R1
Gentile, D; Lichtenstein, G; Rogers, S; Segal, S1
Bercu, BB; Chen, YT; Mattison, DR; Schulman, JD1
Kawakami, M; Misuma, H; Ninomiya, H; Shohmori, T; Wada, H1
Levy, HL; Pacholec, F; Paigen, K1
Fujimura, Y; Kawamura, M; Naruse, H1
Fujimura, Y; Ishii, S; Kawamura, M; Naruse, H1
Berry, GT; Gibson, JB; Mazur, AT; Palmieri, MJ; Reynolds, RA; Segal, S1
Gitzelmann, R1
Rosé-Beutler, B; Schlüchter, R; Wiesmann, UN1
Charlwood, J; Clayton, P; Keir, G; Mian, N; Winchester, B1
Fridovich-Keil, JL; Wohlers, TM1
Aebersold, R; Buhler, J; Bumgarner, R; Christmas, R; Eng, JK; Goodlett, DR; Hood, L; Ideker, T; Ranish, JA; Thorsson, V1
Chen, J; Reynolds, R; Segal, S; Wehrli, SL; Yager, C1
Marinou, K; Schulpis, KH; Tsakiris, S1
Elsas, LJ; Kennedy, MJ; Singh, RH; Webb, AL1
Elsas, LJ; Khwaja, FW; Lai, K; Langley, SD; Schmitt, EW1
Holden, HM; Rayment, I; Thoden, JB1
GOLBERG, L; SCHWARZ, Y1
BURTON, RM; KALCKAR, HM; MAXWELL, ES1
ANDERSON, EP; ISSELBACHER, KJ; KALCKAR, HM1
SEGAL, S; WEINBERG, AN1
BERGREN, WR; DONNELL, GN; WONGIN, NG1
BERGEN, WR; DONNELL, GN; NG, WG1
KIRKMAN, HN; MAXWELL, ES1
Bandyopadhyay, S; Banerjee, S; Chakrabarti, J; Chakravarty, BN; Goswami, SK; Kabir, SN; Pal, AK1
Hammen, HW; Kamalanathan, L; Schadewaldt, P; Wendel, U1
Behrakis, P; Marinou, K; Schulpis, KH; Tsakiris, S1
Bakker, HD; Bosch, AM; Wanders, RJ; Wenniger-Prick, LJ; Wijburg, FA1
Bolouri, H; de Atauri, P; Orrell, D; Ramsey, S1
Behrakis, P; Marinou, K; Schulpis, KH; Tsakiris, S; Tsopanakis, C2
Packman, S; Zlatunich, CO1
Addo, F; Lai, K; Slepak, T; Tang, M1
Michelakakis, H; Schulpis, KH; Tsakiris, S1
Dai, N; Katzir, N; Petreikov, M; Pharr, DM; Portnoy, V; Schaffer, AA1
Brunetti-Pierri, N; Craigen, WJ; Opekun, AR1
Ficicioglu, C; Hussa, C; Segal, S; Yager, C1
Bartzeliotou, A; Kalogerakou, M; Karikas, GA; Papakonstantinou, ED; Schulpis, KH; Tsakiris, S1
Chhay, JS; Eaton, JS; Fridovich-Keil, JL; Mumma, JO; Newell-Litwa, KA; Ross, KL1
Fortes, FS; Martins, LF; Masuda, CA; Mendonça-Previato, L; Montero-Lomelí, M; Previato, JO1
Bro, C; de Jongh, WA; Nielsen, J; Olsson, L; Ostergaard, S; Regenberg, B1
Cuthbert, C; Elsas, L; Klapper, H1
Assis, LJ; Masuda, CA; Mendonça-Previato, L; Miranda, MN; Montero-Lomelí, M; Penha, LL; Previato, JO1
Boers, GH; Bosch, AM; Guion, N; Hammen, HW; Janssen, M; Kamalanathan, L; Schadewaldt, P; Schwarz, M; Wendel, U1
Hong, SP; Jeong, JS; Kwon, HJ; Lee, YM; Yoon, HR1
Clark, A; Geoghegan, O; Hendroff, U; Hughes, J; Lambert, D; Monavari, A; Rogers, Y; Ryan, S; Treacy, EP; Twomey, E1
Elsas, LJ; Lai, K; Wierenga, KJ1
Jun, SH; Kim, JQ; Ko, DH; Park, HD; Park, KU; Song, J; Song, SH; Song, YH1
Fernhoff, PM1
Ficicioglu, C; Gallagher, PR; Hussa, C; Thomas, N; Yager, C1
Berry, GT1
DuBoff, B; Feany, MB; Fridovich-Keil, JL; Ryan, EL1
Fridovich-Keil, JL; Hang, D; Hopson, ML; Jones, DP; Jumbo-Lucioni, PP; Liang, Y1
Cardoso, MT; Coelho, AI; Costa, C; de Almeida, IT; Diogo, L; Ferreira, E; Garcia, P; Gaspar, A; Leite, M; Martins, E; Oliveira, A; Paiva, S; Ramos, R; Rivera, I; Rodrigues, E; Sequeira, S; Silva, MJ; Teles, EL; Vicente, JB1
Al-Essa, M; Al-Qabandi, W; Dhaunsi, GS; Khan, I1
da-Silva, WS; De-Souza, EA; Machado, CM; Martins, LS; Masuda, CA; Montero-Lomelí, M; Pimentel, FS1
Daenzer, JM; Fridovich-Keil, JL; Garza, KR; Hopson, ML; Jumbo-Lucioni, PP; Ryan, EL1
Abu-Soud, HM; Aldhaheri, SR; Gonik, B; Jeelani, R; Khan, SN; Kohan-Ghadr, HR; Shaeib, F; Thakur, M1
Ammon, A; Dillner, B; Grün, N; Höll, C; Kämper, J; Klebl, F; Schuler, D; Ulrich, J; Voll, LM1
An, D; Balakrishnan, B; DeAntonis, C; Lai, K; Martini, PGV; Nguyen, V1
Cheng, LY; Hagen, T; Henke, W; J Zhou, JF; Oh, SL1
Daenzer, JMI; Fridovich-Keil, JL; Geurts, AM; Head, ST; MacWilliams, JA; Rasmussen, SA; Schroeder, JP; Weinshenker, D; Williams, MB1
Camara, H; De-Queiroz, ALFV; De-Souza, EA; Felix-Formiga, ML; Galina, A; Machado, CM; Masuda, CA; Montero-Lomeli, M; Mori, MA; Pimentel, FSA; Pinto, S1
Druss, JJ; Fridovich-Keil, JL; Potter, NL; Rudd Zhong Manis, J1

Reviews

4 review(s) available for galactose-1-phosphate and galactose

ArticleYear
Galactose-1-phosphate in the pathophysiology of galactosemia.
    European journal of pediatrics, 1995, Volume: 154, Issue:7 Suppl 2

    Topics: Galactose; Galactosemias; Galactosephosphates; Glucose; Humans; Infant, Newborn; Phosphorylation

1995
Structure and function of enzymes of the Leloir pathway for galactose metabolism.
    The Journal of biological chemistry, 2003, Nov-07, Volume: 278, Issue:45

    Topics: Adenosine Triphosphate; Binding Sites; Carbohydrate Epimerases; Galactokinase; Galactose; Galactosemias; Galactosephosphates; Humans; Models, Molecular; Phosphorylation; UDPglucose 4-Epimerase; Uridine Diphosphate Galactose; Uridine Diphosphate Glucose; UTP-Hexose-1-Phosphate Uridylyltransferase

2003
Galactose toxicity in animals.
    IUBMB life, 2009, Volume: 61, Issue:11

    Topics: Animals; Galactitol; Galactokinase; Galactose; Galactosemias; Galactosephosphates; Humans; Infant, Newborn; Inositol; Models, Animal; Neonatal Screening; UDPglucose 4-Epimerase; UTP-Hexose-1-Phosphate Uridylyltransferase

2009
Galactosemia: when is it a newborn screening emergency?
    Molecular genetics and metabolism, 2012, Volume: 106, Issue:1

    Topics: Galactose; Galactosemias; Galactosephosphates; Genotype; Humans; Infant, Newborn; Mutation; Neonatal Screening; Polymorphism, Genetic; UTP-Hexose-1-Phosphate Uridylyltransferase

2012

Trials

2 trial(s) available for galactose-1-phosphate and galactose

ArticleYear
Leguminosae in the diet: the raffinose-stachyose question.
    European journal of pediatrics, 1995, Volume: 154, Issue:7 Suppl 2

    Topics: Adult; Child; Fabaceae; Female; Galactose; Galactosemias; Galactosephosphates; Humans; Oligosaccharides; Plants, Medicinal; Raffinose

1995
Verbal dyspraxia and galactosemia.
    Pediatric research, 2003, Volume: 53, Issue:3

    Topics: Adult; Apraxias; Breath Tests; Carbon Dioxide; Carbon Isotopes; Child; Erythrocytes; Female; Galactitol; Galactose; Galactosemias; Galactosephosphates; Genotype; Humans; Male; Phenotype; Prospective Studies; Reproducibility of Results; Risk Factors; Sensitivity and Specificity

2003

Other Studies

70 other study(ies) available for galactose-1-phosphate and galactose

ArticleYear
[Clinical and biochemical diagnosis of galactosemia among our cases].
    Problemy medycyny wieku rozwojowego, 1979, Volume: 8

    Topics: Adult; Blood Glucose; Erythrocytes; Female; Galactose; Galactosemias; Galactosephosphates; Humans; Infant; Infant, Newborn; Male; Pedigree; UDPglucose-Hexose-1-Phosphate Uridylyltransferase; UTP-Hexose-1-Phosphate Uridylyltransferase

1979
A study of galactose intolerance in human and rat liver in vivo by 31P magnetic resonance spectroscopy.
    Pediatric research, 1992, Volume: 32, Issue:1

    Topics: Adenosine Triphosphate; Adult; Animals; Child; Disease Models, Animal; Female; Galactose; Galactosemias; Galactosephosphates; Humans; Liver; Magnetic Resonance Spectroscopy; Male; NAD; Phosphates; Rats; Rats, Inbred Strains; Uridine Diphosphate Galactose

1992
Culture of galactosaemic fibroblasts in the presence of galactose: effect of inosine.
    Journal of inherited metabolic disease, 1990, Volume: 13, Issue:6

    Topics: Cell Division; Cells, Cultured; Child, Preschool; Culture Media; Fibroblasts; Galactokinase; Galactose; Galactosemias; Galactosephosphates; Humans; Infant; Inosine; UTP-Hexose-1-Phosphate Uridylyltransferase

1990
Results of newborn screening for galactose metabolic disorders.
    Journal of inherited metabolic disease, 1990, Volume: 13, Issue:1

    Topics: Carbohydrate Metabolism, Inborn Errors; Galactose; Galactosemias; Galactosephosphates; Genetic Carrier Screening; Humans; Infant, Newborn; Mass Screening; UDPglucose 4-Epimerase

1990
Galactosaemia: sensitive bioluminescent assays for galactose and red cell galactose-1-phosphate.
    Journal of inherited metabolic disease, 1990, Volume: 13, Issue:3

    Topics: Erythrocytes; Galactose; Galactosemias; Galactosephosphates; Humans; Luciferases; Luminescent Measurements

1990
Newborn screening for galactosemia: a new method used in Manitoba.
    Pediatrics, 1989, Volume: 84, Issue:2

    Topics: Galactose; Galactosemias; Galactosephosphates; Humans; Infant, Newborn; Manitoba; Mass Screening

1989
Metabolic studies of a sustained galactose overload in rat.
    Annals of nutrition & metabolism, 1987, Volume: 31, Issue:6

    Topics: Animals; Diet; Disease Models, Animal; Galactitol; Galactokinase; Galactose; Galactosephosphates; Liver; Male; Nucleotidyltransferases; Rats; Rats, Inbred Strains; UDPglucose-Hexose-1-Phosphate Uridylyltransferase

1987
The binding of decomposition products of UDP-galactose to the microsomes and polyribosomes isolated from rat liver.
    Biochemical medicine and metabolic biology, 1987, Volume: 38, Issue:2

    Topics: Animals; Carbon Radioisotopes; Galactose; Galactosephosphates; Glycoproteins; Glycosylation; In Vitro Techniques; Microsomes, Liver; Polyribosomes; Rats; Uridine Diphosphate Galactose; Uridine Diphosphate Sugars

1987
Microassay for estimation of galactose and galactose-1-phosphate in dried blood specimens.
    Clinical biochemistry, 1986, Volume: 19, Issue:4

    Topics: Alkaline Phosphatase; Blood Stains; Fluorometry; Galactose; Galactose Dehydrogenases; Galactosemias; Galactosephosphates; Hexosephosphates; Humans; Infant, Newborn; Microchemistry

1986
Oral intake of glucose plus galactose and erythrocyte galactose-1-phosphate. A nutritional evaluation of hydrolyzed lactose.
    Zeitschrift fur Ernahrungswissenschaft, 1986, Volume: 25, Issue:3

    Topics: Adult; Erythrocytes; Female; Galactose; Galactosemias; Galactosephosphates; Glucose; Glucose Solution, Hypertonic; Hexosephosphates; Humans; Male; Middle Aged; Risk

1986
Regulation and characterization of the galactose-phosphoenolpyruvate-dependent phosphotransferase system in Lactobacillus casei.
    Journal of bacteriology, 1983, Volume: 154, Issue:3

    Topics: Adenosine Triphosphate; Enzyme Induction; Galactokinase; Galactose; Galactosephosphates; Lacticaseibacillus casei; Phosphoenolpyruvate Sugar Phosphotransferase System; Phosphorylation; Substrate Specificity

1983
Further observations in a case of uridine diphosphate galactose-4-epimerase deficiency with a severe clinical presentation.
    Journal of inherited metabolic disease, 1983, Volume: 6, Issue:1

    Topics: Biotransformation; Carbohydrate Epimerases; Dietary Carbohydrates; Erythrocytes; Female; Galactose; Galactosemias; Galactosephosphates; Humans; Infant; Infant, Newborn; UDPglucose 4-Epimerase; Uridine Diphosphate Galactose

1983
Accumulation of galactonate in liver of suckling rats perfused with galactose.
    Biochemical and biophysical research communications, 1984, Jan-13, Volume: 118, Issue:1

    Topics: Animals; Animals, Suckling; Galactose; Galactosephosphates; Kinetics; Liver; Rats; Rats, Inbred Strains; Sugar Acids

1984
Resistance of the male gonad to a high galactose diet.
    Pediatric research, 1984, Volume: 18, Issue:4

    Topics: Animals; Body Weight; Drug Resistance; Female; Fetus; Galactose; Galactosephosphates; Gonadotropins; Male; Organ Size; Ovary; Pregnancy; Rats; Rats, Inbred Strains; Testis; Testosterone

1984
Galactose and galactose-1-phosphate spot test for galactosemia screening.
    Clinica chimica acta; international journal of clinical chemistry, 1981, Mar-19, Volume: 111, Issue:1

    Topics: Escherichia coli; Fluorescence; Galactose; Galactosemias; Galactosephosphates; Hexosephosphates; Humans; NAD

1981
A new method of screening for inherited disorders of galactose metabolism.
    The Journal of laboratory and clinical medicine, 1982, Volume: 99, Issue:6

    Topics: Bacteriophages; Escherichia coli; Galactose; Galactosemias; Galactosephosphates; Hexosephosphates; Humans; Infant, Newborn; Mass Screening; Microbiological Techniques; Mutation

1982
Simultaneous quantitative estimation of galactose-1-phosphate and galactose in blood for the diagnosis of galactosemia.
    The Tohoku journal of experimental medicine, 1982, Volume: 137, Issue:3

    Topics: Alkaline Phosphatase; Galactose; Galactose Dehydrogenases; Galactosemias; Galactosephosphates; Hexosephosphates; Humans; Infant; Methods

1982
Microdetermination of galactose and galactose 1-phosphate in dried blood spots.
    Analytical biochemistry, 1981, Volume: 117, Issue:1

    Topics: Adult; Autoanalysis; Drug Stability; Galactose; Galactosemias; Galactosephosphates; Hemoglobins; Hexosephosphates; Humans; Infant, Newborn; Microchemistry; Protein Denaturation; Substrate Specificity

1981
Effect of glucose and galactose loading in normal subjects on red and white blood cell uridine diphosphate sugars.
    Biochemical and molecular medicine, 1995, Volume: 55, Issue:1

    Topics: Administration, Oral; Adult; Blood Glucose; Dietary Carbohydrates; Erythrocytes; Female; Galactose; Galactosemias; Galactosephosphates; Glucose; Glycosuria; Humans; Leukocytes; Male; Middle Aged; Uridine Diphosphate Galactose; Uridine Diphosphate Glucose

1995
Defective galactosylation of serum transferrin in galactosemia.
    Glycobiology, 1998, Volume: 8, Issue:4

    Topics: Carbohydrate Sequence; Chromatography, High Pressure Liquid; Dietary Carbohydrates; Erythrocytes; Galactose; Galactosemias; Galactosephosphates; Glycosylation; Humans; Molecular Sequence Data; Molecular Structure; Polysaccharides; Transferrin; UDPglucose-Hexose-1-Phosphate Uridylyltransferase

1998
Studies of the V94M-substituted human UDPgalactose-4-epimerase enzyme associated with generalized epimerase-deficiency galactosaemia.
    Journal of inherited metabolic disease, 2000, Volume: 23, Issue:7

    Topics: Amino Acid Substitution; Galactose; Galactosemias; Galactosephosphates; Gene Expression; Humans; Kinetics; Methionine; Saccharomyces cerevisiae; UDPglucose 4-Epimerase; Uridine Diphosphate N-Acetylgalactosamine; Valine

2000
Integrated genomic and proteomic analyses of a systematically perturbed metabolic network.
    Science (New York, N.Y.), 2001, May-04, Volume: 292, Issue:5518

    Topics: Computational Biology; Culture Media; Databases, Factual; Fungal Proteins; Galactose; Galactosephosphates; Gene Expression Profiling; Gene Expression Regulation, Fungal; Genome, Fungal; Models, Biological; Models, Genetic; Monosaccharide Transport Proteins; Mutation; Oligonucleotide Array Sequence Analysis; Proteome; RNA, Fungal; RNA, Messenger; Saccharomyces cerevisiae; Saccharomyces cerevisiae Proteins

2001
Galactose metabolism in normal human lymphoblasts studied by (1)H, (13)C and (31)P NMR spectroscopy of extracts.
    NMR in biomedicine, 2001, Volume: 14, Issue:3

    Topics: Carbon Isotopes; Cell Extracts; Cells, Cultured; Galactitol; Galactose; Galactosephosphates; Humans; Hydrogen; Lymphocytes; Magnetic Resonance Spectroscopy; Phosphorus Isotopes; Uridine Diphosphate Galactose; Uridine Diphosphate Glucose

2001
The in vitro effects of galactose and its derivatives on rat brain Mg2+-ATPase activity.
    Pharmacology & toxicology, 2002, Volume: 91, Issue:5

    Topics: Animals; Animals, Suckling; Brain; Ca(2+) Mg(2+)-ATPase; Dose-Response Relationship, Drug; Enzyme Activation; Female; Galactitol; Galactose; Galactosephosphates; Glucose; Glucosephosphates; In Vitro Techniques; Male; Rats; Rats, Wistar

2002
GALT deficiency causes UDP-hexose deficit in human galactosemic cells.
    Glycobiology, 2003, Volume: 13, Issue:4

    Topics: Cell Line, Transformed; Cell Transformation, Viral; Cells, Cultured; Fibroblasts; Galactose; Galactosephosphates; Glycosylation; Humans; Kinetics; Phenotype; Simian virus 40; Transfection; Uridine Diphosphate Glucose; UTP-Glucose-1-Phosphate Uridylyltransferase; UTP-Hexose-1-Phosphate Uridylyltransferase

2003
Galactose 1-phosphate in galactose cataract.
    Biochimica et biophysica acta, 1955, Volume: 18, Issue:2

    Topics: Cataract; Galactose; Galactosephosphates; Hexosephosphates; Humans; Lens, Crystalline; Phosphates

1955
Galacto-waldenase and the enzymic incorporation of galactose 1-phosphate in mammalian tissues.
    Biochimica et biophysica acta, 1955, Volume: 18, Issue:3

    Topics: Animals; Galactose; Galactosephosphates; Hexosephosphates; Isomerases; Mammals; Phosphates

1955
Defect in uptake of galactose-1-phosphate into liver nucleotides in congenital galactosemia.
    Science (New York, N.Y.), 1957, Jan-18, Volume: 125, Issue:3238

    Topics: Biochemical Phenomena; Galactose; Galactosemias; Galactosephosphates; Humans; Liver; Nucleotides

1957
Effect of galactose-1-phosphate on glucose oxidation by normal and galactosemic leukocytes.
    Science (New York, N.Y.), 1960, Oct-14, Volume: 132, Issue:3433

    Topics: Animals; Carbohydrate Metabolism; Galactose; Galactosephosphates; Glucose; Glucosephosphate Dehydrogenase; Lens, Crystalline; Leukocytes; Oxidation-Reduction; Phosphates; Rats

1960
GALACTOSE-1-PHOSPHATE URIDYL TRANSFERASE ACTIVITY IN GALACTOSAEMIA.
    Nature, 1964, Aug-22, Volume: 203

    Topics: Carbon Dioxide; Clinical Enzyme Tests; Erythrocytes; Galactose; Galactosemias; Galactosephosphates; Genetics, Medical; Humans; Leukocytes; Metabolism; Nucleotidyltransferases; UDPglucose-Hexose-1-Phosphate Uridylyltransferase

1964
GALACTOSE-1-PHOSPHATE URIDYLTRANSFERASE ASSAY BY USE OF RADIOACTIVE GALACTOSE-1-PHOSPHATE.
    Clinica chimica acta; international journal of clinical chemistry, 1964, Volume: 10

    Topics: Biological Assay; Blood; Down Syndrome; Galactose; Galactosemias; Galactosephosphates; Hexosephosphates; Humans; Nucleotidyltransferases; UTP-Hexose-1-Phosphate Uridylyltransferase

1964
Enzymatic estimation of erythrocytic galactose-1-phosphate.
    The Journal of laboratory and clinical medicine, 1960, Volume: 56

    Topics: Enzymes; Erythrocytes; Galactose; Galactosephosphates; Humans; Regression Analysis

1960
Galactose toxicity in the rat as a model for premature ovarian failure: an experimental approach readdressed.
    Human reproduction (Oxford, England), 2003, Volume: 18, Issue:10

    Topics: Aging; Animals; Animals, Newborn; Estradiol; Female; Galactose; Galactosephosphates; Gonadotropins; Ovary; Ovulation Induction; Pregnancy; Prenatal Exposure Delayed Effects; Primary Ovarian Insufficiency; Rats; Rats, Sprague-Dawley; Superovulation; Vagina

2003
Stable-isotope dilution analysis of galactose metabolites in human erythrocytes.
    Rapid communications in mass spectrometry : RCM, 2003, Volume: 17, Issue:24

    Topics: Blood Chemical Analysis; Erythrocytes; Female; Galactitol; Galactose; Galactosemias; Galactosephosphates; Gas Chromatography-Mass Spectrometry; Humans; Isotope Labeling; Male; Metabolic Clearance Rate; Radioisotope Dilution Technique; Reproducibility of Results; Sensitivity and Specificity

2003
Protective effect of L-cysteine and glutathione on the modulated suckling rat brain Na+, K+, -ATPase and Mg2+ -ATPase activities induced by the in vitro galactosaemia.
    Pharmacological research, 2004, Volume: 49, Issue:5

    Topics: Animals; Animals, Suckling; Antioxidants; Brain; Brain Diseases, Metabolic, Inborn; Ca(2+) Mg(2+)-ATPase; Cysteine; Drug Combinations; Drug Evaluation, Preclinical; Female; Galactitol; Galactose; Galactosemias; Galactosephosphates; Glutathione; Male; Rats; Rats, Wistar; Sodium-Potassium-Exchanging ATPase

2004
High tolerance for oral galactose in classical galactosaemia: dietary implications.
    Archives of disease in childhood, 2004, Volume: 89, Issue:11

    Topics: Administration, Oral; Adolescent; Drug Tolerance; Female; Fruit; Galactitol; Galactose; Galactosemias; Galactosephosphates; Humans; Male; Vegetables

2004
Is the regulation of galactose 1-phosphate tuned against gene expression noise?
    The Biochemical journal, 2005, Apr-01, Volume: 387, Issue:Pt 1

    Topics: Galactose; Galactosephosphates; Gene Expression Regulation, Fungal; Kinetics; Protein Biosynthesis; RNA, Fungal; RNA, Messenger; Saccharomyces cerevisiae; Saccharomyces cerevisiae Proteins; Transcription, Genetic; UTP-Hexose-1-Phosphate Uridylyltransferase

2005
Mg2+-ATPase activity in suckling rat brain regions in galactosaemia in vitro. L-Cysteine and glutathione effects.
    Toxicology in vitro : an international journal published in association with BIBRA, 2005, Volume: 19, Issue:2

    Topics: Animals; Animals, Suckling; Antioxidants; Brain; Ca(2+) Mg(2+)-ATPase; Cysteine; Drug Combinations; Enzyme Activation; Female; Galactitol; Galactose; Galactosemias; Galactosephosphates; Glutathione; Male; Rats; Rats, Wistar

2005
Galactosaemia: early treatment with an elemental formula.
    Journal of inherited metabolic disease, 2005, Volume: 28, Issue:2

    Topics: Erythrocytes; Female; Food, Formulated; Galactose; Galactosemias; Galactosephosphates; Humans; Infant Formula; Infant, Newborn; Infant, Newborn, Diseases

2005
Suckling rat brain regional distribution of Na+,K+-atpase activity in the in vitro galactosaemia: the effect of L-cysteine and glutathione.
    Metabolic brain disease, 2005, Volume: 20, Issue:1

    Topics: Animals; Animals, Suckling; Antioxidants; Brain; Brain Chemistry; Cysteine; Disease Models, Animal; Enzyme Activation; Female; Free Radicals; Galactitol; Galactose; Galactosemias; Galactosephosphates; Glutathione; In Vitro Techniques; Male; Oxidative Stress; Rats; Rats, Wistar; Sodium-Potassium-Exchanging ATPase; Subcellular Fractions

2005
Intracellular galactose-1-phosphate accumulation leads to environmental stress response in yeast model.
    Molecular genetics and metabolism, 2005, Volume: 86, Issue:3

    Topics: Environment; Galactokinase; Galactose; Galactosemias; Galactosephosphates; Gene Expression Profiling; Humans; Models, Biological; Mutation; Oligonucleotide Array Sequence Analysis; Saccharomyces cerevisiae; UTP-Hexose-1-Phosphate Uridylyltransferase

2005
Erythrocyte membrane acetylcholinesterase, Na+, K+-ATPase and Mg2+-ATPase activities in patients with classical galactosaemia.
    Acta paediatrica (Oslo, Norway : 1992), 2005, Volume: 94, Issue:9

    Topics: Acetylcholinesterase; Ca(2+) Mg(2+)-ATPase; Child; Erythrocyte Membrane; Galactose; Galactosemias; Galactosephosphates; Greece; Humans; Hydrolases; Sodium-Potassium-Exchanging ATPase

2005
Cloning and expression analysis of a UDP-galactose/glucose pyrophosphorylase from melon fruit provides evidence for the major metabolic pathway of galactose metabolism in raffinose oligosaccharide metabolizing plants.
    Plant physiology, 2006, Volume: 142, Issue:1

    Topics: Amino Acid Sequence; Cloning, Molecular; Cucumis melo; Fruit; Galactose; Galactosephosphates; Gene Expression Regulation, Developmental; Gene Expression Regulation, Plant; Glucosephosphates; Molecular Sequence Data; Nucleotidyltransferases; Raffinose; Sequence Analysis, DNA; Sequence Analysis, Protein; Sequence Homology, Amino Acid; UDPglucose-Hexose-1-Phosphate Uridylyltransferase

2006
Two familial cases of high blood galactose of unknown aetiology.
    Journal of inherited metabolic disease, 2006, Volume: 29, Issue:6

    Topics: Carbohydrate Metabolism, Inborn Errors; Cell Membrane; Chromosomes, Human, X; Family Health; Galactitol; Galactose; Galactosemias; Galactosephosphates; Humans; Infant, Newborn; Lactose; Male

2006
Effect of galactose free formula on galactose-1-phosphate in two infants with classical galactosemia.
    European journal of pediatrics, 2008, Volume: 167, Issue:5

    Topics: Female; Follow-Up Studies; Galactose; Galactosemias; Galactosephosphates; Humans; Infant Formula; Infant, Newborn; Male

2008
The effect of diet on Paraoxonase 1/Arylesterase activities in patients with disorders of galactose metabolism.
    Clinical endocrinology, 2007, Volume: 67, Issue:5

    Topics: Antioxidants; Apolipoprotein A-I; Aryldialkylphosphatase; Carboxylic Ester Hydrolases; Case-Control Studies; Child; Diet; Follow-Up Studies; Galactose; Galactosemias; Galactosephosphates; Humans; Lipids; Lipoproteins; Spectrophotometry

2007
Distinct roles of galactose-1P in galactose-mediated growth arrest of yeast deficient in galactose-1P uridylyltransferase (GALT) and UDP-galactose 4'-epimerase (GALE).
    Molecular genetics and metabolism, 2008, Volume: 93, Issue:2

    Topics: Doxycycline; Galactokinase; Galactose; Galactosephosphates; Gene Deletion; Gene Expression Regulation, Fungal; Genes, Fungal; Saccharomyces cerevisiae; UDPglucose 4-Epimerase; UDPglucose-Hexose-1-Phosphate Uridylyltransferase

2008
Lithium-mediated suppression of morphogenesis and growth in Candida albicans.
    FEMS yeast research, 2008, Volume: 8, Issue:4

    Topics: Antifungal Agents; Candida albicans; Culture Media; Galactose; Galactosephosphates; Gene Deletion; Glucosephosphates; Hyphae; Inhibitory Concentration 50; Lithium; Magnesium; Phosphoglucomutase; Protein Phosphatase 2; Saccharomyces cerevisiae Proteins; Sirolimus

2008
The roles of galactitol, galactose-1-phosphate, and phosphoglucomutase in galactose-induced toxicity in Saccharomyces cerevisiae.
    Biotechnology and bioengineering, 2008, Oct-01, Volume: 101, Issue:2

    Topics: Culture Media; Galactitol; Galactokinase; Galactose; Galactosephosphates; Multienzyme Complexes; Phosphoglucomutase; Plasmids; Saccharomyces cerevisiae; Saccharomyces cerevisiae Proteins; Trans-Activators

2008
Diagnosis of inherited disorders of galactose metabolism.
    Current protocols in human genetics, 2008, Volume: Chapter 17

    Topics: Carbohydrate Metabolism, Inborn Errors; DNA Mutational Analysis; DNA Primers; Galactitol; Galactokinase; Galactose; Galactosemias; Galactosephosphates; Genetics, Medical; Humans; Infant, Newborn; Neonatal Screening; Polymerase Chain Reaction; UDPglucose 4-Epimerase; UTP-Hexose-1-Phosphate Uridylyltransferase

2008
Overexpression of the aldose reductase GRE3 suppresses lithium-induced galactose toxicity in Saccharomyces cerevisiae.
    FEMS yeast research, 2008, Volume: 8, Issue:8

    Topics: Aldehyde Reductase; Antimanic Agents; Galactokinase; Galactose; Galactosephosphates; Gene Deletion; Gene Expression Regulation, Fungal; Lithium; Saccharomyces cerevisiae; Saccharomyces cerevisiae Proteins; Up-Regulation

2008
Biochemical monitoring of pregnancy and breast feeding in five patients with classical galactosaemia--and review of the literature.
    European journal of pediatrics, 2009, Volume: 168, Issue:6

    Topics: Adult; Breast Feeding; Cesarean Section; Erythrocytes; Female; Galactitol; Galactose; Galactosemias; Galactosephosphates; Humans; Lactation; Pregnancy; Pregnancy Complications; Pregnancy Outcome; Young Adult

2009
Galactosemia screening by simultaneous blood spot quantification of galactose and galactose 1-phosphate.
    Clinical chemistry, 2008, Volume: 54, Issue:12

    Topics: Adult; Anion Exchange Resins; Blood Specimen Collection; Chromatography, Ion Exchange; Galactose; Galactosemias; Galactosephosphates; Humans; Infant, Newborn

2008
Outcomes of siblings with classical galactosemia.
    The Journal of pediatrics, 2009, Volume: 154, Issue:5

    Topics: Adolescent; Adult; Birth Order; Brain; Child; Child, Preschool; Diet Records; Female; Galactitol; Galactose; Galactosemias; Galactosephosphates; Humans; Infant; Intelligence Tests; Ireland; Lactose; Language Disorders; Magnetic Resonance Spectroscopy; Male; Neurologic Examination; Patient Compliance; Retrospective Studies; Siblings; Young Adult

2009
Multiplex enzyme assay for galactosemia using ultraperformance liquid chromatography-tandem mass spectrometry.
    Clinical chemistry, 2010, Volume: 56, Issue:5

    Topics: Chromatography, High Pressure Liquid; Enzyme Assays; Erythrocytes; Galactokinase; Galactose; Galactosemias; Galactosephosphates; Humans; Linear Models; Reproducibility of Results; Tandem Mass Spectrometry; UDPglucose 4-Epimerase; UTP-Hexose-1-Phosphate Uridylyltransferase

2010
Duarte galactosemia: how sweet is it?
    Clinical chemistry, 2010, Volume: 56, Issue:7

    Topics: Child; Child, Preschool; Dietary Carbohydrates; Galactitol; Galactose; Galactosemias; Galactosephosphates; Humans; Infant; Sugar Acids

2010
Monitoring of biochemical status in children with Duarte galactosemia: utility of galactose, galactitol, galactonate, and galactose 1-phosphate.
    Clinical chemistry, 2010, Volume: 56, Issue:7

    Topics: Child; Child, Preschool; Dietary Carbohydrates; Erythrocytes; Female; Galactitol; Galactose; Galactosemias; Galactosephosphates; Humans; Infant; Male; Monitoring, Physiologic; Reference Values; Sugar Acids

2010
Mediators of a long-term movement abnormality in a Drosophila melanogaster model of classic galactosemia.
    Disease models & mechanisms, 2012, Volume: 5, Issue:6

    Topics: Aging; Animals; Brain; Diet; Disease Models, Animal; Drosophila melanogaster; Drosophila Proteins; Dyskinesias; Galactose; Galactosemias; Galactosephosphates; Galactosyltransferases; Humans; Motor Activity; Movement; Muscles; Reflex, Startle; Time Factors

2012
Oxidative stress contributes to outcome severity in a Drosophila melanogaster model of classic galactosemia.
    Disease models & mechanisms, 2013, Volume: 6, Issue:1

    Topics: Animals; Antioxidants; Ascorbic Acid; Cysteine; Dimethyl Sulfoxide; Disease Models, Animal; Drosophila melanogaster; Drosophila Proteins; Galactose; Galactosemias; Galactosephosphates; Gene Expression; Gene Knockout Techniques; Genes, Insect; Glutathione; Glutathione Transferase; Humans; Mutation; Oxidative Stress; Paraquat; Reactive Oxygen Species; UDPglucose-Hexose-1-Phosphate Uridylyltransferase; Xanthones

2013
A frequent splicing mutation and novel missense mutations color the updated mutational spectrum of classic galactosemia in Portugal.
    Journal of inherited metabolic disease, 2014, Volume: 37, Issue:1

    Topics: Adolescent; Adult; Alleles; DNA Mutational Analysis; Female; Galactose; Galactosemias; Galactosephosphates; Gene Frequency; Genetic Association Studies; Homozygote; Humans; Male; Mutation, Missense; Phenotype; Portugal; RNA Splicing; UTP-Hexose-1-Phosphate Uridylyltransferase; Young Adult

2014
Impaired NADPH oxidase activity in peripheral blood lymphocytes of galactosemia patients.
    Experimental biology and medicine (Maywood, N.J.), 2013, Volume: 238, Issue:7

    Topics: Antioxidants; Blotting, Western; Case-Control Studies; Cell Separation; Cells, Cultured; Child, Preschool; Galactose; Galactosemias; Galactosephosphates; Glucosephosphate Dehydrogenase; Humans; Infant; Lipid Peroxidation; Lymphocytes; Malondialdehyde; NADPH Oxidase 1; NADPH Oxidases; Nitrites; Phosphoproteins

2013
The unfolded protein response has a protective role in yeast models of classic galactosemia.
    Disease models & mechanisms, 2014, Volume: 7, Issue:1

    Topics: Alternative Splicing; Basic-Leucine Zipper Transcription Factors; Endoplasmic Reticulum; Fungal Proteins; Galactokinase; Galactose; Galactosemias; Galactosephosphates; Gene Expression Regulation, Fungal; Glycoproteins; HSP70 Heat-Shock Proteins; Humans; Mutation; Oxidoreductases Acting on Sulfur Group Donors; Protein Folding; Repressor Proteins; RNA, Messenger; Saccharomyces cerevisiae; Saccharomyces cerevisiae Proteins; Unfolded Protein Response

2014
Acute and long-term outcomes in a Drosophila melanogaster model of classic galactosemia occur independently of galactose-1-phosphate accumulation.
    Disease models & mechanisms, 2016, 11-01, Volume: 9, Issue:11

    Topics: Animals; Disease Models, Animal; Drosophila melanogaster; Female; Fertility; Galactokinase; Galactose; Galactosemias; Galactosephosphates; Larva; Male; Metabolic Networks and Pathways; Phenotype

2016
Galactose and its Metabolites Deteriorate Metaphase II Mouse Oocyte Quality and Subsequent Embryo Development by Disrupting the Spindle Structure.
    Scientific reports, 2017, 03-22, Volume: 7, Issue:1

    Topics: Animals; Embryonic Development; Galactitol; Galactose; Galactosephosphates; Metaphase; Mice; Oocytes; Spindle Apparatus

2017
Galactose metabolism and toxicity in Ustilago maydis.
    Fungal genetics and biology : FG & B, 2018, Volume: 114

    Topics: Amino Acid Sequence; Fungal Proteins; Galactokinase; Galactose; Galactosephosphates; Gene Expression Regulation, Fungal; Genes, Fungal; Metabolic Networks and Pathways; Mutagenesis; Sequence Deletion; Ustilago

2018
Novel mRNA-Based Therapy Reduces Toxic Galactose Metabolites and Overcomes Galactose Sensitivity in a Mouse Model of Classic Galactosemia.
    Molecular therapy : the journal of the American Society of Gene Therapy, 2020, 01-08, Volume: 28, Issue:1

    Topics: Animals; Animals, Newborn; Cells, Cultured; Disease Models, Animal; Erythrocytes; Female; Fibroblasts; Galactose; Galactosemias; Galactosephosphates; Humans; Injections, Intraperitoneal; Injections, Intravenous; Liver; Male; Mice; Mice, Knockout; RNA, Messenger; Signal Transduction; Transfection; Treatment Outcome; UTP-Hexose-1-Phosphate Uridylyltransferase

2020
Galactose 1-phosphate accumulates to high levels in galactose-treated cells due to low GALT activity and absence of product inhibition of GALK.
    Journal of inherited metabolic disease, 2020, Volume: 43, Issue:3

    Topics: Galactose; Galactosemias; Galactosephosphates; HEK293 Cells; Humans; UTP-Hexose-1-Phosphate Uridylyltransferase

2020
A galactose-1-phosphate uridylyltransferase-null rat model of classic galactosemia mimics relevant patient outcomes and reveals tissue-specific and longitudinal differences in galactose metabolism.
    Journal of inherited metabolic disease, 2020, Volume: 43, Issue:3

    Topics: Animals; Animals, Newborn; Disease Models, Animal; Female; Galactose; Galactosemias; Galactosephosphates; Male; Phenotype; Rats; Rats, Sprague-Dawley; UTP-Hexose-1-Phosphate Uridylyltransferase

2020
The yeast protein Ubx4p contributes to mitochondrial respiration and lithium-galactose-mediated activation of the unfolded protein response.
    The Journal of biological chemistry, 2020, 03-20, Volume: 295, Issue:12

    Topics: Basic-Leucine Zipper Transcription Factors; Endoplasmic Reticulum; Galactose; Galactosephosphates; Intracellular Signaling Peptides and Proteins; Lithium; Mitochondria; Oxygen Consumption; Protein Serine-Threonine Kinases; Repressor Proteins; RNA Splicing; Saccharomyces cerevisiae; Saccharomyces cerevisiae Proteins; Unfolded Protein Response

2020
Grip strength in patients with galactosemia and in a galactose-1-phosphate uridylyltransferase (GALT)-null rat model.
    Journal of inherited metabolic disease, 2023, Volume: 46, Issue:6

    Topics: Animals; Female; Galactose; Galactosemias; Hand Strength; Humans; Male; Rats; UTP-Hexose-1-Phosphate Uridylyltransferase

2023