Galactose 1-phosphate |
| Catalog No.: GC30337 |
Galactose 1-phosphate is an intermediate in the galactose metabolism and nucleotide sugars.
Sample solution is provided at 25 µL, 10mM.
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Quality Control & SDS
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Purity: >98.00%
- COA (Certificate Of Analysis)
- SDS (Safety Data Sheet)
- Datasheet
| Cell experiment [1]: | |
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Cell lines |
Fibroblasts derived from the foreskin of 3-8-day-old healthy neonates |
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Preparation Method |
Galactose 1-phosphate (Gal-1-P) at different concentrations (1-16 mM) was preincubated separately with whole brain homogenates at 37℃ for 1 h. |
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Reaction Conditions |
0-10 mM；1-14 days |
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Applications |
Gal-1-P can stimulated rat brain Na+,K+-ATPase. |
| Animal experiment [2]: | |
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Animal models |
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Preparation Method |
Cells were cultured for 1-14 days with 0-10 mM Gal-1-P and then stimulated with 5% fetal bovine serum (FBS) or 50 ng/mL of platelet-derived growth factor (PDGF) or fibroblast growth factor (FGF) or IGF-1 for 24 h. |
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Dosage form |
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Applications |
Gal-1-P (5-10 mM) in culture medium for 7-14 days significantly decreased IGF-1-, PDGF-and FBS-stimulated DNA synthesis. |
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References: [1]. Tsakiris S, Marinou K,et,al. The effect of galactose metabolic disorders on rat brain Na+,K+-ATPase activity. Z Naturforsch C J Biosci. 2002 Sep-Oct;57(9-10):939-43. doi: 10.1515/znc-2002-9-1030. PMID: 12440737. |
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Galactose 1-phosphate (Gal-1-P) is the substrate of galactose 1-phosphate uridylyltransferase (GALT), which is cytotoxic and has inhibitory effect on many enzymes in glucose metabolism pathway[1,2].Gal-1-P(1-16 mM;37℃；1h) can stimulated rat brain Na+,K+-ATPase[3].
Gal-1-P (5-10 mM) in culture medium for 7-14 days significantly decreased IGF-1, PDGF and FBS-stimulated DNA synthesis in Fibroblasts[4]. Various concentrations (1-16 mM) of galactose-1-phosphate (Gal-1-P) were preincubated with brain homogenates of suckling rats as well as with pure eel Electroforus electricus AChE at 37℃ for 1 h. Gal-1-P decreased gradually the enzyme activity reaching a plateau, when incubated with 8-16 mM. Gal-1-P can affect acetylcholine degradation acting directly on AChE molecule[5].
References:
[1]. Oh SL, Cheng LY, et,al. Galactose 1-phosphate accumulates to high levels in galactose-treated cells due to low GALT activity and absence of product inhibition of GALK. J Inherit Metab Dis. 2020 May;43(3):529-539. doi: 10.1002/jimd.12198. Epub 2019 Dec 17. PMID: 31774565.
[2]. Lai K, Langley SD, et,al. GALT deficiency causes UDP-hexose deficit in human galactosemic cells. Glycobiology. 2003 Apr;13(4):285-94. doi: 10.1093/glycob/cwg033. Epub 2003 Jan 3. PMID: 12626383.
[3]. Tsakiris S, Marinou K, et,al. The effect of galactose metabolic disorders on rat brain Na+,K+-ATPase activity. Z Naturforsch C J Biosci. 2002 Sep-Oct;57(9-10):939-43. doi: 10.1515/znc-2002-9-1030. PMID: 12440737.
[4]. Al-Essa M, Dhaunsi G. Receptor-mediated attenuation of insulin-like growth factor-1 activity by galactose-1-phosphate in neonate skin fibroblast cultures: Galactosemia pathogenesis. Adv Clin Exp Med. 2020 Apr;29(4):499-504. doi: 10.17219/acem/111807. PMID: 32356415.
[5]. Tsakiris S, Schulpis KH. The effect of galactose metabolic disorders on rat brain acetylcholinesterase activity. Z Naturforsch C J Biosci. 2000 Sep-Oct;55(9-10):852-5. doi: 10.1515/znc-2000-9-1032. PMID: 11098845.
| Cas No. | 2255-14-3 | SDF | |
| Canonical SMILES | O=P(O)(O)O[C@H]1O[C@@H]([C@H](O)[C@H](O)[C@H]1O)CO | ||
| Formula | C6H13O9P | M.Wt | 260.14 |
| Solubility | Water:125 mg/mL (371.67 mM) | Storage | Store at -20°C |
| General tips | For obtaining a higher solubility , please warm the tube at 37 ℃ and shake it in the ultrasonic bath for a while.Stock solution can be stored below -20℃ for several months. | ||
| Shipping Condition | Evaluation sample solution : ship with blue ice All other available size: ship with RT , or blue ice upon request |
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Galactose 1 Phosphate Uridyltransferase Deficiency
Galactosemia is an inborn error of metabolism due to impaired degradation of galactose. The disease was first described in 1917, and defective galactose metabolism was explained as the cause in the 1950s. If not recognized and treated promptly, affected infants may develop significant morbidity within days of birth. Newborn screening tests in developed countries have helped identify affected infants early. However, providers must maintain a high index of suspicion in ill newborns as feeding intolerance, hepatomegaly, lethargy, coagulopathy, and renal dysfunction may occur within the first few days of life, even before newborn screening tests have been finalized.
Galactose-1-phosphate uridyltransferase deficiency: A literature review of the putative mechanisms of short and long-term complications and allelic variants
Galactosemia type 1 is an autosomal recessive disorder of galactose metabolism, determined by a deficiency in the enzyme galactose-1-phosphate uridyltransferase (GALT). GALT deficiency is classified as severe or variant depending on biochemical phenotype, genotype and potential to develop acute and long-term complications. Neonatal symptoms usually resolve after galactose-restricted diet; however, some patients, despite the diet, can develop long-term complications, in particular when the GALT enzyme activity results absent or severely decreased. The mechanisms of acute and long-term complications are still discussed and several hypotheses are presented in the literature like enzymatic inhibition, osmotic stress, endoplasmic reticulum stress, oxidative stress, defects of glycosylation or epigenetic modification. This review summarizes the current knowledge of galactosemia, in particular the putative mechanisms of neonatal and long-term complications and the molecular genetics of GALT deficiency.
Galactose-1-phosphate in the pathophysiology of galactosemia
In galactosemia, galactose-1-phosphate (gal-1-P) is not properly metabolized and accumulates in the fetus and after birth in various tissues when lactose or galactose is ingested. Well-treated galactosemics retain a low level of red cell gal-1-P which increases after breaks of diet. The ester is an indicator of the biogenesis of galactose from glucose and has been considered a pathogenic agent by inhibiting enzymes such as glucose-6-phosphatase, glucose-6-phosphate dehydrogenase, phosphoglucomutase, and glycogen phosphorylase, but the evidence remains presumptive. A futile cycle of galactose phosphorylation and dephosphorylation, and the sequestration of phosphorus in gal-1-P are also suspected to play a role in the pathogenesis of galactosemia.
Addition of galactose-1-phosphate measurement enhances newborn screening for classical galactosemia
Galactosemia is an inborn disorder of carbohydrate metabolism of which early detection can prevent severe illness. Although the assay for galactose-1-phosphate uridyltransferase (GALT) enzyme activity has been available since the 1960s, many issues prevented it from becoming universal. In order to develop the Israeli newborn screening pilot algorithm for galactosemia, flow injection analysis tandem mass spectrometry measurement of galactose-1-phosphate in archived dried blood spots from newborns with classical galactosemia, galactosemia variants, epimerase deficiency, and normal controls, was conducted. Out of 431 330 newborns screened during the pilot study (30 months), two with classical galactosemia and four with epimerase deficiency were identified and confirmed. Five false positives and no false negatives were recorded. Following this pilot study, the Israeli final and routine newborn screening algorithm, as recommended by the Advisory Committee to the National Newborn Screening Program, now consists of galactose-1-phosphate measurement integrated into the routine tandem mass spectrometry panel as the first-tier screening test, and GALT enzyme activity as the second-tier performed to identify only newborns suspected to be at risk for classical galactosemia. The GALT enzyme activity cut-off used in the final algorithm was lowered in order to avoid false positives.
Current and Future Treatments for Classic Galactosemia
Type I (classic) galactosemia, galactose 1-phosphate uridylyltransferase (GALT)-deficiency is a hereditary disorder of galactose metabolism. The current therapeutic standard of care, a galactose-restricted diet, is effective in treating neonatal complications but is inadequate in preventing burdensome complications. The development of several animal models of classic galactosemia that (partly) mimic the biochemical and clinical phenotypes and the resolution of the crystal structure of GALT have provided important insights; however, precise pathophysiology remains to be elucidated. Novel therapeutic approaches currently being explored focus on several of the pathogenic factors that have been described, aiming to (i) restore GALT activity, (ii) influence the cascade of events and (iii) address the clinical picture. This review attempts to provide an overview on the latest advancements in therapy approaches.
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