Galactose 1-phosphate
(Synonyms: 半乳糖-1-磷酸) 目录号 : GC30337半乳糖1磷酸是半乳糖-1-磷酸尿苷酰转移酶(GALT)的作用底物,具有细胞毒性,对糖代谢途径中的多种酶有抑制作用.Gal-1-P(1- 16mm;37℃;1h)能刺激大鼠脑Na+,K+-ATP酶。
Cas No.:2255-14-3
Sample solution is provided at 25 µL, 10mM.
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Quality Control & SDS
- View current batch:
- 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.
半乳糖1磷酸是半乳糖-1-磷酸尿苷酰转移酶(GALT)的作用底物,具有细胞毒性,对糖代谢途径中的多种酶有抑制作用[1,2].
Gal-1-P(1- 16mm;37℃;1h)能刺激大鼠脑Na+,K+-ATP酶[3]。Gal-1-P (5-10 mM；7-14天)在培养基中显著降低成纤维细胞中IGF-1、PDGF和FBS刺激的DNA合成[4]。
将不同浓度(1 ~ 16 mM)的半乳糖-1-磷酸(Gal-1-P)与乳鼠脑匀浆及纯电鳗乙酰胆碱(AChE)在37℃下孵育1 h, Gal-1-P在8 ~ 16 mM孵育1 h后, 酶活性逐渐降低至稳定。并且Gal-1-P可直接作用于乙酰胆碱分子[5]。
| Cas No. | 2255-14-3 | SDF | |
| 别名 | 半乳糖-1-磷酸 | ||
| Canonical SMILES | O=P(O)(O)O[C@H]1O[C@@H]([C@H](O)[C@H](O)[C@H]1O)CO | ||
| 分子式 | C6H13O9P | 分子量 | 260.14 |
| 溶解度 | Water:125 mg/mL (371.67 mM) | 储存条件 | Store at -20°C |
| General tips | 请根据产品在不同溶剂中的溶解度选择合适的溶剂配制储备液；一旦配成溶液，请分装保存，避免反复冻融造成的产品失效。 储备液的保存方式和期限：-80°C 储存时，请在 6 个月内使用，-20°C 储存时，请在 1 个月内使用。 为了提高溶解度，请将管子加热至37℃，然后在超声波浴中震荡一段时间。 |
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| Shipping Condition | 评估样品解决方案：配备蓝冰进行发货。所有其他可用尺寸：配备RT，或根据请求配备蓝冰。 | ||
| 制备储备液 | |||
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1 mg | 5 mg | 10 mg |
| 1 mM | 3.8441 mL | 19.2204 mL | 38.4408 mL |
| 5 mM | 0.7688 mL | 3.8441 mL | 7.6882 mL |
| 10 mM | 0.3844 mL | 1.922 mL | 3.8441 mL |
| 第一步：请输入基本实验信息（考虑到实验过程中的损耗，建议多配一只动物的药量） | ||||||||||
| 给药剂量 | mg/kg |  |
动物平均体重 | g | |
每只动物给药体积 | ul | |
动物数量 | 只 |
| 第二步：请输入动物体内配方组成（配方适用于不溶于水的药物；不同批次药物配方比例不同，请联系GLPBIO为您提供正确的澄清溶液配方） | ||||||||||
| % DMSO % % Tween 80 % saline | ||||||||||
| 计算重置 | ||||||||||
计算结果:
工作液浓度： mg/ml；
DMSO母液配制方法： mg 药物溶于 μL DMSO溶液（母液浓度 mg/mL，
体内配方配制方法：取 μL DMSO母液，加入 μL PEG300，混匀澄清后加入μL Tween 80，混匀澄清后加入 μL saline，混匀澄清。
1. 首先保证母液是澄清的；
2.
一定要按照顺序依次将溶剂加入，进行下一步操作之前必须保证上一步操作得到的是澄清的溶液，可采用涡旋、超声或水浴加热等物理方法助溶。
3. 以上所有助溶剂都可在 GlpBio 网站选购。
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.