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1972 2
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Quoted phrase not found in phrase index: "Fructose-biphosphatase deficiency"
Page 1
Hyperglycolysis in endothelial cells drives endothelial injury and microvascular alterations in peritoneal dialysis.
Si Z, Su W, Zhou Z, Li J, Su C, Zhang Y, Hu Z, Huang Z, Zhou H, Cong A, Zhou Z, Cao W. Si Z, et al. Clin Transl Med. 2023 Dec;13(12):e1498. doi: 10.1002/ctm2.1498. Clin Transl Med. 2023. PMID: 38037461 Free PMC article.
This was combined with EC-selective blockade of glycolysis by genetic or pharmacological inhibition of 6-phosphofructo-2-kinase/fructose-2,6-biphosphatase 3 (PFKFB3) in vivo and in vitro. We also investigated the association between peritoneal EC glycolysis and micr …
This was combined with EC-selective blockade of glycolysis by genetic or pharmacological inhibition of 6-phosphofructo-2-kinase/fructose …
Inborn errors of carbohydrate metabolism.
Mayatepek E, Hoffmann B, Meissner T. Mayatepek E, et al. Best Pract Res Clin Gastroenterol. 2010 Oct;24(5):607-18. doi: 10.1016/j.bpg.2010.07.012. Best Pract Res Clin Gastroenterol. 2010. PMID: 20955963 Review.
Glycogen storage diseases (GSD) and inborn errors of galactose and fructose metabolism are the most common representatives of inborn errors of carbohydrate metabolism. ...A lactose-free infant formula can be life-saving in affected neonates whereas a strict fructose …
Glycogen storage diseases (GSD) and inborn errors of galactose and fructose metabolism are the most common representatives of inborn …
Fructose-1,6-diphosphatase deficiency.
Hopwood NJ, Holzman I, Drash AL. Hopwood NJ, et al. Am J Dis Child. 1977 Apr;131(4):418-21. doi: 10.1001/archpedi.1977.02120170044009. Am J Dis Child. 1977. PMID: 192069
A girl aged 3 years and 11 months, with recurrent episodes of unexplained metabolic acidosis, hepatomegaly, and fasting hypoglycemia unresponsive to glucagon, showed profound falls in blood glucose levels in response to oral fructose and glycerol challenge. In vitro analys …
A girl aged 3 years and 11 months, with recurrent episodes of unexplained metabolic acidosis, hepatomegaly, and fasting hypoglycemia unrespo …
Intravenous glycerol therapy should not be used in patients with unrecognized fructose-1,6-bisphosphatase deficiency.
Hasegawa Y, Kikawa Y, Miyamaoto J, Sugimoto S, Adachi M, Ohura T, Mayumi M. Hasegawa Y, et al. Pediatr Int. 2003 Feb;45(1):5-9. doi: 10.1046/j.1442-200x.2003.01662.x. Pediatr Int. 2003. PMID: 12654061
BACKGROUND: In Asian countries, glycerol solution that contains fructose (5%) is often used for management of brain edema. However, glycerol and fructose may cause severe hypoglycemia and metabolic acidosis in patients with fructose-1,6-bisphosphatase (FBPase …
BACKGROUND: In Asian countries, glycerol solution that contains fructose (5%) is often used for management of brain edema. However, g …
Hepatic fructose-1,6-diphosphatase deficiency. A cause of lactic acidosis and hypoglycemia in infancy.
Pagliara AS, Karl IE, Keating JP, Brown BI, Kipnis DM. Pagliara AS, et al. J Clin Invest. 1972 Aug;51(8):2115-23. doi: 10.1172/JCI107018. J Clin Invest. 1972. PMID: 4341015 Free PMC article.
Hepatic glycolytic intermediates were increased below the level of fructose-1,6-diphosphate and decreased above. Hepatic phosphorylase, glucose-6-phosphatase, amylo-1,6-glucosidase, phosphofructokinase, fructose-1-phosphate aldolase, and fructose-1,6-diphosph …
Hepatic glycolytic intermediates were increased below the level of fructose-1,6-diphosphate and decreased above. Hepatic phosphorylas …
Fructose-1,6-diphosphatase deficiency: a treatable neurometabolic disorder.
Kamate M, Jambagi M, Gowda P, Sonoli S. Kamate M, et al. BMJ Case Rep. 2014 Sep 22;2014:bcr2013201553. doi: 10.1136/bcr-2013-201553. BMJ Case Rep. 2014. PMID: 25246452 Free PMC article.
Fructose-1,6-diphosphatase (FDPase) deficiency is usually considered an inborn error of fructose metabolism, however, strictly speaking it is a defect of gluconeogenesis. The disorder is manifested by the appearance of hypoglycaemia, keto …
Fructose-1,6-diphosphatase (FDPase) deficiency is usually considered an inborn error of fructose m …
Novel fructose-1,6-bisphosphatase gene mutation in two siblings.
Eren E, Edgunlu T, Abuhandan M, Yetkin I. Eren E, et al. DNA Cell Biol. 2013 Nov;32(11):635-9. doi: 10.1089/dna.2013.2119. Epub 2013 Sep 5. DNA Cell Biol. 2013. PMID: 24007283
Fructose-1,6-bisphosphatase (FBPase) deficiency is an autosomal, recessively inherited disease that progresses with severe hypoglycemia, and metabolic attacks result in a defect in gluconeogenesis. If not appropriately treated, and if fructose is not excluded …
Fructose-1,6-bisphosphatase (FBPase) deficiency is an autosomal, recessively inherited disease that progresses with severe hyp …
Atherogenic Lipoprotein(a) Increases Vascular Glycolysis, Thereby Facilitating Inflammation and Leukocyte Extravasation.
Schnitzler JG, Hoogeveen RM, Ali L, Prange KHM, Waissi F, van Weeghel M, Bachmann JC, Versloot M, Borrelli MJ, Yeang C, De Kleijn DPV, Houtkooper RH, Koschinsky ML, de Winther MPJ, Groen AK, Witztum JL, Tsimikas S, Stroes ESG, Kroon J. Schnitzler JG, et al. Circ Res. 2020 May 8;126(10):1346-1359. doi: 10.1161/CIRCRESAHA.119.316206. Epub 2020 Mar 12. Circ Res. 2020. PMID: 32160811 Free PMC article.
Transcriptome analysis of Lp(a)-stimulated human arterial endothelial cells revealed upregulation of inflammatory pathways comprising monocyte adhesion and migration, coinciding with increased 6-phophofructo-2-kinase/fructose-2,6-biphosphatase (PFKFB)-3-mediated gly …
Transcriptome analysis of Lp(a)-stimulated human arterial endothelial cells revealed upregulation of inflammatory pathways comprising monocy …
Changes of liver metabolite concentrations in adults with disorders of fructose metabolism after intravenous fructose by 31P magnetic resonance spectroscopy.
Boesiger P, Buchli R, Meier D, Steinmann B, Gitzelmann R. Boesiger P, et al. Pediatr Res. 1994 Oct;36(4):436-40. doi: 10.1203/00006450-199410000-00004. Pediatr Res. 1994. PMID: 7816517 Clinical Trial.
We have validated this approach by measuring ATP, phosphomonesters, and inorganic phosphate (Pi) during fasting and after an i.v. fructose bolus in healthy adults and in three adults with disorders of fructose metabolism and by comparing results with known metabolic …
We have validated this approach by measuring ATP, phosphomonesters, and inorganic phosphate (Pi) during fasting and after an i.v. fructos …
18 results