Galactitol
Galactitol is a naturally occurring product of plants obtained following reduction of galactose. It appears as a white crystalline powder with a slight sweet taste. It may form in excess in the lens of the eye in galactosemias a deficiency of galactokinase.
Structure for HMDB00107 (Galactitol)
C6H14O6
182.1718
182.07903818
(2R,3S,4R,5S)-hexane-1,2,3,4,5,6-hexol
galactitol
608-66-2
FBPFZTCFMRRESA-GUCUJZIJSA-N
Not classified
Detected and Quantified
Solid
GC-MS Spectrum – GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies)splash10-014j-0941000000-61141f9f2ddc66e0b016View in MoNA
GC-MS Spectrum – GC-MS (6 TMS)splash10-0gb9-1983000000-0a0dafcfea843fbb3c72View in MoNA
LC-MS/MS Spectrum – Quattro_QQQ 10V, N/A (Annotated)splash10-005a-5900000000-35da0de23de5b9170aedView in MoNA
LC-MS/MS Spectrum – Quattro_QQQ 25V, N/A (Annotated)splash10-0a4i-9000000000-552b84764ceb9b497a7eView in MoNA
LC-MS/MS Spectrum – Quattro_QQQ 40V, N/A (Annotated)splash10-052f-9000000000-dfb4eef021d94d514588View in MoNA
1H NMR SpectrumNot Available
13C NMR SpectrumNot Available
[1H,13C] 2D NMR SpectrumNot Available
Normal
Normal
Galactosemia
Irritable bowel syndrome
Galactosemia
- Shetty HU, Holloway HW, Schapiro MB: Cerebrospinal fluid and plasma distribution of myo-inositol and other polyols in Alzheimer disease. Clin Chem. 1996 Feb;42(2):298-302. [PubMed:8595727 ]
- Jakobs C, Schweitzer S, Dorland B: Galactitol in galactosemia. Eur J Pediatr. 1995;154(7 Suppl 2):S50-2. [PubMed:7671965 ]
- Ning C, Segal S: Plasma galactose and galactitol concentration in patients with galactose-1-phosphate uridyltransferase deficiency galactosemia: determination by gas chromatography/mass spectrometry. Metabolism. 2000 Nov;49(11):1460-6. [PubMed:11092512 ]
- Jakobs C, Warner TG, Sweetman L, Nyhan WL: Stable isotope dilution analysis of galactitol in amniotic fluid: an accurate approach to the prenatal diagnosis of galactosemia. Pediatr Res. 1984 Aug;18(8):714-8. [PubMed:6433315 ]
Not Available
Not Available
Not Available
Not Available
FDB006453
C00001160
11357
C01697
15-DIDEOXY-15-IMINO-D-GALACTITOL
38183
Galactitol
HMDB00107
HMDB00107
5148
11850
Not Available
16813
- Ning C, Segal S: Plasma galactose and galactitol concentration in patients with galactose-1-phosphate uridyltransferase deficiency galactosemia: determination by gas chromatography/mass spectrometry. Metabolism. 2000 Nov;49(11):1460-6. [PubMed:11092512 ]
- Jakobs C, Warner TG, Sweetman L, Nyhan WL: Stable isotope dilution analysis of galactitol in amniotic fluid: an accurate approach to the prenatal diagnosis of galactosemia. Pediatr Res. 1984 Aug;18(8):714-8. [PubMed:6433315 ]
- Ning C, Reynolds R, Chen J, Yager C, Berry GT, McNamara PD, Leslie N, Segal S: Galactose metabolism by the mouse with galactose-1-phosphate uridyltransferase deficiency. Pediatr Res. 2000 Aug;48(2):211-7. [PubMed:10926297 ]
- Budde M, Gusek-Schneider GC, Junemann A, Jansen F, Shin YS: [Familial cataract in plasma galactitol increase without known enzyme defect]. Klin Monbl Augenheilkd. 1999 Oct;215(4):255-7. [PubMed:10572890 ]
- Berry GT, Hunter JV, Wang Z, Dreha S, Mazur A, Brooks DG, Ning C, Zimmerman RA, Segal S: In vivo evidence of brain galactitol accumulation in an infant with galactosemia and encephalopathy. J Pediatr. 2001 Feb;138(2):260-2. [PubMed:11174626 ]
- Pettit BR, King GS, Blau K: The analysis of hexitols in biological fluid by selected ion monitoring. Biomed Mass Spectrom. 1980 Jul;7(7):309-13. [PubMed:7448335 ]
- Ficicioglu C, Yager C, Segal S: Galactitol and galactonate in red blood cells of children with the Duarte/galactosemia genotype. Mol Genet Metab. 2005 Feb;84(2):152-9. Epub 2004 Dec 9. [PubMed:15670721 ]
- Jakobs C, Schweitzer S, Dorland B: Galactitol in galactosemia. Eur J Pediatr. 1995;154(7 Suppl 2):S50-2. [PubMed:7671965 ]
- Roboz J, Kappatos DC, Greaves J, Holland JF: Determination of polyols in serum by selected ion monitoring. Clin Chem. 1984 Oct;30(10):1611-5. [PubMed:6434200 ]
- Airey CM, Price DE, Kemp JV, Perkins CM, Wales JK: The effect of aldose reductase inhibition on erythrocyte polyols and galactitol accumulation in diabetic patients. Diabet Med. 1989 Dec;6(9):804-8. [PubMed:2533041 ]
- Schwarz HP, Schaefer T, Bachmann C: Galactose and galactitol in the urine of children with compound heterozygosity for Duarte variant and classical galactosemia (GtD/gt) after an oral galactose load. Clin Chem. 1985 Mar;31(3):420-2. [PubMed:3971562 ]
- Arola H, Sillanaukee P, Aine E, Koivula T, Isokoski M: Galactitol is not a cause of senile cataract. Graefes Arch Clin Exp Ophthalmol. 1992;230(3):240-2. [PubMed:1597290 ]
- Allen JT, Holton JB, Gillett MG: Gas-liquid chromatographic determination of galactitol in amniotic fluid for possible use in prenatal diagnosis of galactosaemia. Clin Chim Acta. 1981 Feb 19;110(1):59-63. [PubMed:7214715 ]
- Shetty HU, Holloway HW, Rapoport SI: Capillary gas chromatography combined with ion trap detection for quantitative profiling of polyols in cerebrospinal fluid and plasma. Anal Biochem. 1995 Jan 1;224(1):279-85. [PubMed:7710082 ]
- Berry GT, Palmieri M, Gross KC, Acosta PB, Henstenburg JA, Mazur A, Reynolds R, Segal S: The effect of dietary fruits and vegetables on urinary galactitol excretion in galactose-1-phosphate uridyltransferase deficiency. J Inherit Metab Dis. 1993;16(1):91-100. [PubMed:8487507 ]
Enzymes
- General function:
- Involved in oxidoreductase activity
- Specific function:
- Catalyzes the NADPH-dependent reduction of a wide variety of carbonyl-containing compounds to their corresponding alcohols with a broad range of catalytic efficiencies.
- Gene Name:
- AKR1B1
- Uniprot ID:
- P15121
- Molecular weight:
- 35853.125
Reactions
- General function:
- Not Available
- Specific function:
- Acts as all-trans-retinaldehyde reductase. Can efficiently reduce aliphatic and aromatic aldehydes, and is less active on hexoses (in vitro). May be responsible for detoxification of reactive aldehydes in the digested food before the nutrients are passed on to other organs.
- Gene Name:
- AKR1B10
- Uniprot ID:
- O60218
- Molecular weight:
- Not Available