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Hereditary fructose inInborn error of fructose metabolismAsymptomatic until they ingest fructoseGlucoseDeficiencyDihydroxyacetoneReversibleIngestionHepaticIsozymesMalabsorptionLiverSucroseEnzymaticAccumulationIntoleranceDietaryMetabolicHypoglycemiaPeptidesFructokinaseSymptomsMolecularCellularDiagnosisImmunoassaySensitivityComparingTherapeuticHumanTestsApplications

Hereditary fructose in10

• Within the next 4-5 years, the enzyme defect in aldolase B isozyme in the liver was demonstrated, and hereditary fructose intolerance (HFI) became recognized as a distinct clinical entity. (medscape.com)
• The absence of the latter function readily explains the clinical hypoglycemia in individuals with hereditary fructose intolerance. (medscape.com)
• However, the imperative to making a correct diagnosis distinction is the ongoing hepatic damage seen in hereditary fructose intolerance (HFI), even in the presence of trace amounts of dietary fructose. (medscape.com)
• The spectrum of aldolase B (ALDOB) mutations and the prevalence of hereditary fructose intolerance in Central Europe. (medscape.com)
• A Novel Frameshift Mutation of the ALDOB Gene in a Korean Girl Presenting with Recurrent Hepatitis Diagnosed as Hereditary Fructose Intolerance. (medscape.com)
• Coffee EM, Tolan DR. Mutations in the promoter region of the aldolase B gene that cause hereditary fructose intolerance. (medscape.com)
• Tolan DR. Molecular basis of hereditary fructose intolerance: mutations and polymorphisms in the human aldolase B gene. (medscape.com)
• Hereditary fructose intolerance. (medscape.com)
• Mass RE, Smith WR, Walsh JR. The association of hereditary fructose intolerance and renal tubular acidosis. (medscape.com)
• Hereditary fructose intolerance (HFI) is an inborn error of fructose metabolism caused by a deficiency of the enzyme aldolase B. Individuals affected with HFI are asymptomatic until they ingest fructose, sucrose, or sorbitol. (wikipedia.org)

Inborn error of fructose metabolism1

• An inborn error of fructose metabolism. (medscape.com)

Asymptomatic until they ingest fructose1

• Affected individuals are completely asymptomatic until they ingest fructose. (medscape.com)

Glucose6

• Although lactose is the carbohydrate base in most infant formulas, some (eg, soy formulas) contain sucrose, a fructose-glucose disaccharide that may cause symptoms. (medscape.com)
• Fructose malabsorption and intolerance: effects of fructose with and without simultaneous glucose ingestion. (medscape.com)
• Fructose is replaced in the diet by glucose, maltose or other sugars. (wikipedia.org)
• This process weeded out some previously reported biomarkers and left the Steens with three significant proteins: enzymes involved in energy metabolism, in particular, associated with the breakdown of glucose: fructose-bisphosphate aldolase A, L-lactate dehydrogenase B chain, and pyruvate kinase. (technologynetworks.com)
• 1. Glucose and fructose form similar osazone crystals. (medical-junction.com)
• The vast majority of glucose and fructose entering a cell will become converted to fructose 1,6-phosphate at some point. (wikidoc.org)

Deficiency8

• The following year, researchers reported a familial incidence of the disorder in several family members, postulating that the defect was a deficiency of hepatic fructose 1-aldolase. (medscape.com)
• Thus, in an infant who is homozygous for fructose 1-aldolase deficiency, fructose ingestion triggers a cascade of biochemical events that result in severe clinical disease. (medscape.com)
• This condition is important to distinguish clearly from genetic fructose 1-phosphate aldolase deficiency, although results of breath hydrogen testing are controversial in diagnosis. (medscape.com)
• Glycogen storage disease type XII is a rare metabolic disease resulting from Aldolase A deficiency that causes muscle glycogen accumulation, with crisis of rhabdomyolysis and hemolytic anemia. (biomedcentral.com)
• A case report of a patient with a new phenotype of Aldolase A deficiency characterized by epilepsy and rhabdomyolysis, Treated with ketogenic diet. (biomedcentral.com)
• Glycogen storage disease type XII (GSD XII) is an ultra-rare autosomal recessive disorder caused by aldolase A (ALDOA) deficiency, characterized by hemolytic anemia and rhabdomyolysis with or without myopathy or intellectual disability. (biomedcentral.com)
• The first case of aldolase A deficiency was reported in 1973 by Beutler et al. (biomedcentral.com)
• The proband, was a boy aged 14-years when the diagnosis of aldolase deficiency was established. (biomedcentral.com)

Dihydroxyacetone2

• and condensation of the triose phosphates, glyceraldehyde phosphate, and dihydroxyacetone phosphate to form fructose 1,6-diphosphate). (medscape.com)
• Fructose-1-phosphate is metabolized by aldolase B into dihydroxyacetone phosphate and glyceraldehyde. (wikipedia.org)

Reversible1

• Because the reaction is reversible, aldolase B is an essential enzyme in the process of gluconeogenesis (which is, in some respects, a reversal of glycolysis). (medscape.com)

Ingestion2

• The rapid early progress in the understanding of this disorder may have occurred because of the fairly dramatic and difficult-to-miss symptoms associated with fructose ingestion. (medscape.com)
• After ingestion, fructose is converted to fructose-1-phosphate in the liver by fructokinase. (wikipedia.org)

Hepatic2

• The cause of severe hepatic dysfunction remains unknown but may be a manifestation of focal cytoplasmic degeneration and cellular fructose toxicity. (medscape.com)
• An inborn defect of hepatic fructose-1-phosphate splitting aldolase. (medscape.com)

Isozymes1

• Three aldolase isozymes (A, B, and C) are known encoded by 65 different genes. (biomedcentral.com)

Malabsorption3

• The overlap of gastrointestinal symptoms, such as severe abdominal pain and diarrhea combined with poor growth in young children, has led to reports of a condition known as "fructose intolerance" in association with intestinal fructose malabsorption. (medscape.com)
• It has been postulated that fructose malabsorption may be a consequence of abnormalities in the intestinal GLUT5 transporter, although the results of molecular investigations are inconsistent. (medscape.com)
• Molecular analysis of the fructose transporter gene(GLUT5) in isolated fructose malabsorption. (medscape.com)

Liver1

• If fructose is ingested, the enzymatic block at aldolase B causes an accumulation of fructose-1-phosphate which, over time, results in the death of liver cells. (wikipedia.org)

Sucrose3

• Affected individuals are asymptomatic and healthy, provided they do not ingest foods containing fructose or any of its common precursors, sucrose and sorbitol. (wikipedia.org)
• In the past, infants often became symptomatic when they were introduced to formulas that were sweetened with fructose or sucrose. (wikipedia.org)
• Once identified, parents of infants who carry mutant aldolase B alleles leading to HFI, or older individuals who have clinical histories compatible with HFI can be identified and counselled with regard to preventive therapy: dietary exclusion of foods containing fructose, sucrose, or sorbitol. (wikipedia.org)

Enzymatic1

• In normal cellular conditions, the primary enzymatic activity of aldolase B is to cleave fructose diphosphate (FDP), which forms rather than condenses the triose phosphate compounds. (medscape.com)

Accumulation1

• Reduced cleavage of F-1-P leads to its cellular accumulation and fructokinase inhibition, causing free fructose accumulation in the blood. (medscape.com)

Intolerance3

• Clinical intolerance to fructose was initially described in 1956. (medscape.com)
• Tsampalieros A, Beauchamp J, Boland M, Mack DR. Dietary fructose intolerance in children and adolescents. (medscape.com)
• Fructose intolerance in children presenting with abdominal pain. (medscape.com)

Dietary4

• Thus, homozygous neonates remain clinically well until confronted with dietary sources of fructose. (medscape.com)
• When patients are diagnosed with HFI, a dietary history will often reveal an aversion to fruit and other foods that contain large amounts of fructose. (wikipedia.org)
• Because of the ease of therapy (dietary exclusion of fructose), HFI can be effectively managed if properly diagnosed. (wikipedia.org)
• Stable patients without acute intoxication events are treated by careful dietary planning that avoids fructose and its metabolic precursors. (wikipedia.org)

Metabolic1

• Fructose 1,6-phosphate lies within the glycolysis metabolic pathway and is produced by phosphorylation of fructose 6-phosphate . (wikidoc.org)

Hypoglycemia1

• If possible, individuals who suspect they might have HFI should avoid testing via fructose challenge as the results are non-conclusive for individuals with HFI, and even if the diagnostic administration of fructose is properly controlled, profound hypoglycemia and its sequelae can threaten the patient's well-being. (wikipedia.org)

Peptides2

• Results Homocitrullinated peptides from aldolase and cytokeratin were identified, that stimulated CD8-mediated responses in vivo. (bmj.com)
• Very recently we proposed novel di- and tetra-phenylalanine peptides derivatized with gadolinium complexes as potentials supramolecular diagnostic agents for applications in MRI (Magnetic Resonance Imaging). (cnr.it)

Fructokinase1

• Deficiencies of fructokinase cause essential fructosuria, a clinically benign condition characterized by the excretion of unmetabolized fructose in the urine. (wikipedia.org)

Symptoms2

• Kim Y, Park SC, Wolf BW, Hertzler SR. Combination of erythritol and fructose increases gastrointestinal symptoms in healthy adults. (medscape.com)
• The key identifying feature of HFI is the appearance of symptoms with the introduction of fructose to the diet. (wikipedia.org)

Molecular1

• This suspicion is typically confirmed by molecular analysis Treatment of HFI involves strict avoidance of fructose in the diet. (wikipedia.org)

Cellular1

• Zhanga M, Kataokaa K: Nano-structured composites based on calcium phosphate for cellular delivery of therapeutic and diagnostic agents. (trpv-antagonist.com)

Diagnosis2

• Delineate nursing mindfulness connected to prosaic laboratory and diagnostic testing habituated to in the medical diagnosis of pediatric cancer. (wgc2010.sk)
• Diagnosis is based on history, physical examination, and sometimes laboratory tests and diagnostic imaging. (msdmanuals.com)

Immunoassay2

• Diagnostic accuracy of Augurix COVID-19 IgG serology fast check Goals: To validate the diagnostic accuracy of the Augurix SARS-CoV-2 IgM/IgG fast immunoassay diagnostic check (RDT) for COVID-19. (kits-elisa.com)
• Comparison of Commercial ELISA Kits, a Prototype Multiplex Electrochemoluminescent Assay, and a Multiplex Bead-Based Immunoassay for Detecting a Urine-Based Bladder-Cancer-Associated Diagnostic Signature. (myelisakit.com)

Sensitivity1

• Where recent myocardial infarction is suspected, AST has a diagnostic sensitivity of 96%, with a diagnostic sensitivity of 86% at 12 hours after onset of chest pain. (unitslab.com)

Comparing1

• Comparing the performance of the common used eumycetoma diagnostic tests. (ahmedfahal.net)

Therapeutic1

• Currently there are no objective, easily assessed diagnostic markers for Alzheimer's disease, and no good therapeutic options. (technologynetworks.com)

Human2

• while aldolase C occurs in the central nervous system along with aldolase A which is the isoform most abundantly expressed in the human brain [ 3 , 4 ]. (biomedcentral.com)
• In addition, the homocitrullinated aldolase epitope was also detected in human tumor samples. (bmj.com)

Tests1

• Nursing Central , nursing.unboundmedicine.com/nursingcentral/view/Davis-Lab-and-Diagnostic-Tests/425350/3/Aldolase. (unboundmedicine.com)

Applications1

• Notre objectif est de comprendre la biologie de ces bactéries et de traduire cette connaissance en applications dans la médecine, la gestion environnementale, et les énergies renouvelables. (cea.fr)