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inositol and Classic Galactosemia

inositol has been researched along with Classic Galactosemia in 21 studies

Inositol: An isomer of glucose that has traditionally been considered to be a B vitamin although it has an uncertain status as a vitamin and a deficiency syndrome has not been identified in man. (From Martindale, The Extra Pharmacopoeia, 30th ed, p1379) Inositol phospholipids are important in signal transduction.
inositol : Any cyclohexane-1,2,3,4,5,6-hexol.
1D-chiro-inositol : Belonging to the inositol family of compounds, D-chiro-inositol (DCI) is an isomer of glucose. It is an important secondary messenger in insulin signal transduction.
muco-inositol : An inositol that is cyclohexane-1,2,3,4,5,6-hexol having a (1R,2R,3r,4R,5S,6r)-configuration.

Research Excerpts

ExcerptRelevanceReference
"Cellular accumulation of galactitol has been suggested to cause the apparent dietary-independent, long-term complications in classic galactosemia."4.79The role of polyols in the pathophysiology of hypergalactosemia. ( Berry, GT, 1995)
"Classic Galactosemia (CG) is a devastating inborn error of the metabolism caused by mutations in the GALT gene encoding the enzyme galactose-1 phosphate uridylyltransferase in galactose metabolism."4.12Harnessing the Power of Purple Sweet Potato Color and ( Hagen-Lillevik, S; Hale, G; Johnson, J; Lai, K; Persinger, J; Siddiqi, A, 2022)
"MRI measurement of the retinal oxygenation response to a carbogen challenge appears to be a powerful new and noninvasive approach that may be useful for assessing aspects of pathophysiology underlying the development of diabetic retinopathy in galactosemic rats."3.70Subnormal retinal oxygenation response precedes diabetic-like retinopathy. ( Berkowitz, BA; Frank, RN; Hohman, TC; Kern, TS; Kowluru, RA; Prakash, M, 1999)
" Yet, if this metabolic pathway is perturbed due to congenital deficiencies of the three associated enzymes, or an overwhelming presence of galactose, this monosaccharide which is abundantly present in milk and many non-dairy foodstuffs, will become highly toxic to humans and animals."2.45Galactose toxicity in animals. ( Elsas, LJ; Lai, K; Wierenga, KJ, 2009)
"Lens galactitol levels were reduced approximately 57% in 10-day galactose-fed rats treated with the ARI (tolrestat, 100 mg/kg bwt/day, 6-day pretreatment); the changes in the lower segment of glycolysis, alpha-glycerophosphate levels, redox state of NAD-couples, and energy metabolism were partially prevented while NADP+/ NADPH ratios were unchanged and galactose 1-phosphate levels were further increased."1.30Glycolytic pathway, redox state of NAD(P)-couples and energy metabolism in lens in galactose-fed rats: effect of an aldose reductase inhibitor. ( Burgan, J; Faller, A; Obrosova, I; Ostrow, E; Williamson, JR, 1997)
"At 8 months galactosemia reduced NCV to 58% of control values, while ARI-treatment for 8 months improved NCV to 71% of control values."1.29Galactosemia produces ARI-preventable nodal changes similar to those of diabetic neuropathy. ( Basso, M; Cherian, PV; Hohman, TC; Kamijo, M; Sima, AA, 1994)
" One group of dogs was untreated while three other groups were orally dosed with 0."1.28The prevention of biochemical changes in lens, retina, and nerve of galactosemic dogs by the aldose reductase inhibitor AL01576. ( Brazzell, RK; Chandler, ML; Dickerson, JE; Lou, MF; York, BM, 1989)
"Galactitol is a component of normal amniotic fluid with a mean concentration of 0."1.27Stable isotope dilution analysis of galactitol in amniotic fluid: an accurate approach to the prenatal diagnosis of galactosemia. ( Jakobs, C; Nyhan, WL; Sweetman, L; Warner, TG, 1984)
"Both diabetic hyperglycemia and galactosemia caused the prolongation of peak latencies and in some cases a reduction in the amplitudes of oscillatory potentials on the b-wave."1.27The development of electroretinogram abnormalities and the possible role of polyol pathway activity in diabetic hyperglycemia and galactosemia. ( Fujimori, S; Hirata, Y; Okada, K; Segawa, M, 1988)
"A more mild degree of galactosemia, induced by 5 or 21 days of feeding a diet containing 10% galactose to nondiabetic rats, provoked an increase in nerve water content associated with polyol levels of a similar order to those seen in diabetes."1.27Does galactose feeding provide a valid model of consequences of exaggerated polyol-pathway flux in peripheral nerve in experimental diabetes? ( Lambourne, JE; Tomlinson, DR; Willars, GB, 1987)

Research

Studies (21)

TimeframeStudies, this research(%)All Research%
pre-199012 (57.14)18.7374
1990's7 (33.33)18.2507
2000's1 (4.76)29.6817
2010's0 (0.00)24.3611
2020's1 (4.76)2.80

Authors

AuthorsStudies
Hagen-Lillevik, S1
Johnson, J1
Siddiqi, A1
Persinger, J1
Hale, G1
Lai, K2
Elsas, LJ1
Wierenga, KJ1
WELLS, WW2
PITTMAN, TA1
WELLS, HJ1
EGAN, TJ1
Jakobs, C1
Warner, TG1
Sweetman, L1
Nyhan, WL1
Yue, DK1
Hanwell, MA1
Satchell, PM1
Handelsman, DJ1
Turtle, JR1
Berry, GT1
Kamijo, M1
Basso, M1
Cherian, PV1
Hohman, TC2
Sima, AA1
Obrosova, I1
Faller, A1
Burgan, J1
Ostrow, E1
Williamson, JR1
Takemoto, DJ1
Berkowitz, BA1
Kowluru, RA1
Frank, RN1
Kern, TS2
Prakash, M1
Radhakrishnamurthy, B1
Berenson, GS1
Pargaonkar, PS1
Voors, AW1
Srinivasan, SR1
Plavidal, F1
Dolan, P1
Dalferes, ER1
Tomlinson, DR2
Filliatreau, G1
Figliomeni, B1
Hassig, R1
Di Giamberardino, L1
Willars, GB2
Engerman, RL1
Brivet, M1
Migayron, F1
Roger, J1
Cheron, G1
Lemonnier, A1
Lou, MF1
Dickerson, JE1
Chandler, ML1
Brazzell, RK1
York, BM1
Miwa, I1
Kanbara, M1
Wakazono, H1
Okuda, J1
Segawa, M1
Hirata, Y1
Fujimori, S1
Okada, K1
Lambourne, JE1
McIntyre, JP1
Schlichter, DJ1
Wacholtz, MC1
Spieker, SE1
Van Heyningen, R1
Broekhuyse, RM1

Reviews

3 reviews available for inositol and Classic Galactosemia

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

    Topics: Animals; Galactitol; Galactokinase; Galactose; Galactosemias; Galactosephosphates; Humans; Infant, N

2009
The role of polyols in the pathophysiology of hypergalactosemia.
    European journal of pediatrics, 1995, Volume: 154, Issue:7 Suppl 2

    Topics: Animals; Galactitol; Galactosemias; Humans; Inositol

1995
Galactose cataract: a review.
    Experimental eye research, 1971, Volume: 11, Issue:3

    Topics: Age Factors; Alcohol Oxidoreductases; Aminoisobutyric Acids; Animals; Cataract; Dairy Products; Gala

1971

Other Studies

18 other studies available for inositol and Classic Galactosemia

ArticleYear
Harnessing the Power of Purple Sweet Potato Color and
    International journal of molecular sciences, 2022, Aug-04, Volume: 23, Issue:15

    Topics: Animals; Color; Female; Galactose; Galactosemias; Inositol; Ipomoea batatas; Mice; UTP-Hexose-1-Phos

2022
THE ISOLATION AND IDENTIFICATION OF GALACTITOL FROM THE BRAINS OF GALACTOSEMIA PATIENTS.
    The Journal of biological chemistry, 1965, Volume: 240

    Topics: Alcohols; Brain; Brain Chemistry; Carbohydrate Metabolism; Chromatography; Electrophoresis; Galactit

1965
Stable isotope dilution analysis of galactitol in amniotic fluid: an accurate approach to the prenatal diagnosis of galactosemia.
    Pediatric research, 1984, Volume: 18, Issue:8

    Topics: Amniotic Fluid; Female; Galactitol; Galactosemias; Gas Chromatography-Mass Spectrometry; Humans; Ino

1984
The effects of aldose reductase inhibition on nerve sorbitol and myoinositol concentrations in diabetic and galactosemic rats.
    Metabolism: clinical and experimental, 1984, Volume: 33, Issue:12

    Topics: Aldehyde Reductase; Animals; Diabetes Mellitus, Experimental; Erythrocytes; Female; Galactosemias; I

1984
Galactosemia produces ARI-preventable nodal changes similar to those of diabetic neuropathy.
    Diabetes research and clinical practice, 1994, Volume: 25, Issue:2

    Topics: Aldehyde Reductase; Animals; Axons; Body Weight; Diabetic Neuropathies; Fructose; Galactitol; Galact

1994
Glycolytic pathway, redox state of NAD(P)-couples and energy metabolism in lens in galactose-fed rats: effect of an aldose reductase inhibitor.
    Current eye research, 1997, Volume: 16, Issue:1

    Topics: Aldehyde Reductase; Animals; Energy Metabolism; Enzyme Inhibitors; Galactitol; Galactose; Galactosem

1997
Decreases in Raf-1 levels in galactosaemic lens epithelial cells are partially reversed by myo-inositol.
    Acta diabetologica, 1998, Volume: 35, Issue:3

    Topics: Animals; Cattle; Cell Division; Cells, Cultured; DNA; Epithelial Cells; Galactose; Galactosemias; In

1998
Subnormal retinal oxygenation response precedes diabetic-like retinopathy.
    Investigative ophthalmology & visual science, 1999, Volume: 40, Issue:9

    Topics: Animals; Blood Glucose; Carbon Dioxide; Diabetic Retinopathy; Fructose; Galactitol; Galactose; Galac

1999
Serum-free and protein-bound sugars and cardiovascular complications in diabetes mellitus.
    Laboratory investigation; a journal of technical methods and pathology, 1976, Volume: 34, Issue:2

    Topics: Adult; Age Factors; Aged; Analysis of Variance; Blood Glucose; Blood Pressure; Cardiovascular Diseas

1976
Proteins of slow axonal transport in sciatic motoneurones of rats with streptozotocin-induced diabetes or galactosaemia.
    Diabetes research and clinical practice, 1990, Volume: 9, Issue:1

    Topics: Animals; Autoradiography; Axonal Transport; Carbohydrates; Diabetes Mellitus, Experimental; Electrop

1990
Retinal polyol and myo-inositol in galactosemic dogs given an aldose-reductase inhibitor.
    Investigative ophthalmology & visual science, 1991, Volume: 32, Issue:13

    Topics: Aldehyde Reductase; Alloxan; Animals; Chromatography, Gas; Diabetes Mellitus, Experimental; Disease

1991
Lens hexitols and cataract formation during lactation in a woman heterozygote for galactosaemia.
    Journal of inherited metabolic disease, 1989, Volume: 12 Suppl 2

    Topics: Adult; Cataract; Female; Galactitol; Galactokinase; Galactosemias; Genetic Carrier Screening; Humans

1989
The prevention of biochemical changes in lens, retina, and nerve of galactosemic dogs by the aldose reductase inhibitor AL01576.
    Journal of ocular pharmacology, 1989,Fall, Volume: 5, Issue:3

    Topics: Administration, Oral; Aldehyde Reductase; Analysis of Variance; Animals; Dogs; Fluorenes; Galactitol

1989
Analysis of sorbitol, galactitol, and myo-inositol in lens and sciatic nerve by high-performance liquid chromatography.
    Analytical biochemistry, 1988, Aug-15, Volume: 173, Issue:1

    Topics: Animals; Chromatography, High Pressure Liquid; Diabetes Mellitus, Experimental; Galactitol; Galactos

1988
The development of electroretinogram abnormalities and the possible role of polyol pathway activity in diabetic hyperglycemia and galactosemia.
    Metabolism: clinical and experimental, 1988, Volume: 37, Issue:5

    Topics: Animals; Cataract; Diabetes Mellitus, Experimental; Electroretinography; Galactitol; Galactosemias;

1988
Does galactose feeding provide a valid model of consequences of exaggerated polyol-pathway flux in peripheral nerve in experimental diabetes?
    Diabetes, 1987, Volume: 36, Issue:12

    Topics: Animals; Blood Glucose; Body Water; Body Weight; Diabetes Mellitus, Experimental; Galactosemias; Ino

1987
Studies on myo-inositol metabolism in galactosemia.
    Annals of the New York Academy of Sciences, 1969, Oct-17, Volume: 165, Issue:2

    Topics: Adenosine Triphosphate; Aging; Alcohols; Alkaline Phosphatase; Animals; Brain; Carbon Isotopes; Diet

1969
Changes in myo-inositol permeability in the lens due to cataractous conditions.
    Biochimica et biophysica acta, 1968, Sep-17, Volume: 163, Issue:2

    Topics: Alcohols; Animals; Cataract; Diabetes Complications; Female; Galactosemias; Inositol; Lens, Crystall

1968