FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2034853733> ?p ?o ?g. }

• W2034853733 endingPage "773" @default.
• W2034853733 startingPage "765" @default.
• W2034853733 abstract "The mature red cell has lost all capability to divide and to synthetize proteins. It is therefore more exposed than any other cells to suffer from genetically determined enzyme defects. A good number of these have now been identified in the red cell. Only those which impair the erythrocyte viability or function will be considered and their contribution to molecular pathology be discussed.o1)The intrinsic nature of the defect, i. e. a qualitative abnormality of the enzyme molecule, has been approached in several enzyme defects, such as glucose-6-phosphate dehydrogenase (G6PD), pyruvate kinase and NADH diaphorase deficiency. In some cases, the qualitative abnormality may also have a quantitative effect.2)The consequence of the enzyme defects on the red cell metabolism is susprisingly poorly understood. If the correlation between the lack of methemoglobin reductase and methemoglobinemia is easy to assume, no ready explanation can be given to the drug-induced hemolysis associated to the enzyme defects involving NADPH-production and/or glutathione metabolism, nor to the chronic shortage of the red cell life span seen in defects of glycolytic enzymes. Knowing the defective enzyme does not provide an immediate clue to the mechanism of the disorder.3)The extension of the enzyme defect to other blood cells or tissues is variable. In some cases the defect is not shared by the white blood cells (G6PD A-, pyruvate kinase, « regularNADH-diaphorase deficiency, PGM1 deficiency). Sometimes it can be detected in other cells, without extra-hematological signs (G6PD B-, phosphohexose isomerase). At last it can be present in other tissues and give rise to a generalized disease (triose phosphate isomerase deficiency, phosphoglycerate kinase, NADH-diaphorase deficiency with mental retardation). These differences are due to separate genetic control of the enzyme in the red cell (pyruvate kinase), or more often to differences of the in vivo instability of the abnormal enzyme molecule (G6PD, NADH-diaphorase). In this respect the red cell represents a unique tool for investigating molecular ageing of enzymes in eukaryotes. The intrinsic nature of the defect, i. e. a qualitative abnormality of the enzyme molecule, has been approached in several enzyme defects, such as glucose-6-phosphate dehydrogenase (G6PD), pyruvate kinase and NADH diaphorase deficiency. In some cases, the qualitative abnormality may also have a quantitative effect. The consequence of the enzyme defects on the red cell metabolism is susprisingly poorly understood. If the correlation between the lack of methemoglobin reductase and methemoglobinemia is easy to assume, no ready explanation can be given to the drug-induced hemolysis associated to the enzyme defects involving NADPH-production and/or glutathione metabolism, nor to the chronic shortage of the red cell life span seen in defects of glycolytic enzymes. Knowing the defective enzyme does not provide an immediate clue to the mechanism of the disorder. The extension of the enzyme defect to other blood cells or tissues is variable. In some cases the defect is not shared by the white blood cells (G6PD A-, pyruvate kinase, « regularNADH-diaphorase deficiency, PGM1 deficiency). Sometimes it can be detected in other cells, without extra-hematological signs (G6PD B-, phosphohexose isomerase). At last it can be present in other tissues and give rise to a generalized disease (triose phosphate isomerase deficiency, phosphoglycerate kinase, NADH-diaphorase deficiency with mental retardation). These differences are due to separate genetic control of the enzyme in the red cell (pyruvate kinase), or more often to differences of the in vivo instability of the abnormal enzyme molecule (G6PD, NADH-diaphorase). In this respect the red cell represents a unique tool for investigating molecular ageing of enzymes in eukaryotes." @default.
• W2034853733 created "2016-06-24" @default.
• W2034853733 creator A5017616602 @default.
• W2034853733 date "1972-01-01" @default.
• W2034853733 modified "2023-10-18" @default.
• W2034853733 title "Remarques sur les enzymopathies génétiques du globule rouge" @default.
• W2034853733 cites W1510184861 @default.
• W2034853733 cites W1813144815 @default.
• W2034853733 cites W1971810535 @default.
• W2034853733 cites W1998598808 @default.
• W2034853733 cites W2014186596 @default.
• W2034853733 cites W2016626105 @default.
• W2034853733 cites W2024817429 @default.
• W2034853733 cites W2036241464 @default.
• W2034853733 cites W2044477551 @default.
• W2034853733 cites W2052934943 @default.
• W2034853733 cites W2060708334 @default.
• W2034853733 cites W2060724208 @default.
• W2034853733 cites W2073307065 @default.
• W2034853733 cites W2080325881 @default.
• W2034853733 cites W2088671253 @default.
• W2034853733 cites W2089347523 @default.
• W2034853733 cites W2094714679 @default.
• W2034853733 cites W2139535305 @default.
• W2034853733 cites W2199562693 @default.
• W2034853733 cites W2269978667 @default.
• W2034853733 cites W2336731579 @default.
• W2034853733 cites W2337428316 @default.
• W2034853733 cites W2338636335 @default.
• W2034853733 cites W2346263423 @default.
• W2034853733 cites W2395689351 @default.
• W2034853733 cites W2408311375 @default.
• W2034853733 cites W2410383583 @default.
• W2034853733 cites W2410859377 @default.
• W2034853733 cites W2536104122 @default.
• W2034853733 cites W4237666452 @default.
• W2034853733 cites W4300986392 @default.
• W2034853733 doi "https://doi.org/10.1016/s0300-9084(72)80182-2" @default.
• W2034853733 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/4654168" @default.
• W2034853733 hasPublicationYear "1972" @default.
• W2034853733 type Work @default.
• W2034853733 sameAs 2034853733 @default.
• W2034853733 citedByCount "3" @default.
• W2034853733 crossrefType "journal-article" @default.
• W2034853733 hasAuthorship W2034853733A5017616602 @default.
• W2034853733 hasConcept C120763696 @default.
• W2034853733 hasConcept C126322002 @default.
• W2034853733 hasConcept C155911427 @default.
• W2034853733 hasConcept C181199279 @default.
• W2034853733 hasConcept C185592680 @default.
• W2034853733 hasConcept C20251656 @default.
• W2034853733 hasConcept C203014093 @default.
• W2034853733 hasConcept C26865036 @default.
• W2034853733 hasConcept C2776317432 @default.
• W2034853733 hasConcept C2776557347 @default.
• W2034853733 hasConcept C2777208419 @default.
• W2034853733 hasConcept C2778727959 @default.
• W2034853733 hasConcept C2778917026 @default.
• W2034853733 hasConcept C2779902561 @default.
• W2034853733 hasConcept C2780854706 @default.
• W2034853733 hasConcept C55493867 @default.
• W2034853733 hasConcept C71924100 @default.
• W2034853733 hasConcept C86803240 @default.
• W2034853733 hasConceptScore W2034853733C120763696 @default.
• W2034853733 hasConceptScore W2034853733C126322002 @default.
• W2034853733 hasConceptScore W2034853733C155911427 @default.
• W2034853733 hasConceptScore W2034853733C181199279 @default.
• W2034853733 hasConceptScore W2034853733C185592680 @default.
• W2034853733 hasConceptScore W2034853733C20251656 @default.
• W2034853733 hasConceptScore W2034853733C203014093 @default.
• W2034853733 hasConceptScore W2034853733C26865036 @default.
• W2034853733 hasConceptScore W2034853733C2776317432 @default.
• W2034853733 hasConceptScore W2034853733C2776557347 @default.
• W2034853733 hasConceptScore W2034853733C2777208419 @default.
• W2034853733 hasConceptScore W2034853733C2778727959 @default.
• W2034853733 hasConceptScore W2034853733C2778917026 @default.
• W2034853733 hasConceptScore W2034853733C2779902561 @default.
• W2034853733 hasConceptScore W2034853733C2780854706 @default.
• W2034853733 hasConceptScore W2034853733C55493867 @default.
• W2034853733 hasConceptScore W2034853733C71924100 @default.
• W2034853733 hasConceptScore W2034853733C86803240 @default.
• W2034853733 hasIssue "5-6" @default.
• W2034853733 hasLocation W20348537331 @default.
• W2034853733 hasLocation W20348537332 @default.
• W2034853733 hasOpenAccess W2034853733 @default.
• W2034853733 hasPrimaryLocation W20348537331 @default.
• W2034853733 hasRelatedWork W1964899269 @default.
• W2034853733 hasRelatedWork W1981216115 @default.
• W2034853733 hasRelatedWork W2006376204 @default.
• W2034853733 hasRelatedWork W2030801878 @default.
• W2034853733 hasRelatedWork W2085615695 @default.
• W2034853733 hasRelatedWork W2111658541 @default.
• W2034853733 hasRelatedWork W2149137598 @default.
• W2034853733 hasRelatedWork W2317084518 @default.
• W2034853733 hasRelatedWork W2408917317 @default.
• W2034853733 hasRelatedWork W2426963118 @default.
• W2034853733 hasVolume "54" @default.
• W2034853733 isParatext "false" @default.

• next