Matches in SemOpenAlex for { <https://semopenalex.org/work/W1986018588> ?p ?o ?g. }
Showing items 1 to 90 of
90
with 100 items per page.
- W1986018588 endingPage "83" @default.
- W1986018588 startingPage "73" @default.
- W1986018588 abstract "Measurement of delta-aminolaevulinic acid (ALA) synthetase has been performed in bone marrow of 24 normal subjects and 20 patients with sideroblastic anaemia (five congenital, two pyridoxine-responsive, 12 primary acquired and one secondary form due to isoniazid therapy). ALA-synthetase activity without added pyridoxal-phosphate in vitro was decreased in four of the congenital cases, and in all 12 primary acquired cases. In two of these 16 cases with low ALA-synthetase activity and in a case with sideroblastic change secondary to isoniazid therapy, the enzyme activity was corrected to normal by pyridoxal-phosphate in vitro. ALA-synthetase activity was normal in both cases of pyridoxine-responsive anaemia while they were receiving pyridoxine therapy but in one of these cases, studied at repeated intervals after withdrawal of pyridoxine therapy, the bone marrow ALA-synthetase activity fell to very low levels unless pyridoxal-phosphate was added in vitro. In this case the Km of the enzyme for pyridoxal-phosphate was substantially greater than that of a control sample. Haem synthetase activity was reduced in the bone marrow of two of six cases of primary acquired sideroblastic anaemia and in one congenital case tested but was normal in the pyridoxine-responsive patient studied on pyridoxine therapy. Nevertheless, this patient showed reduced incorporation of 14C-labelled delta-aminolaevulinic acid into bone marrow haem. It is concluded that both congenital and primary acquired sideroblastic anaemia are a heterogenous group of disorders, with different defects in haem synthesis, reduced activity of delta-aminolaevulinic acid synthetase being common but not invariable in both types. Pyridoxine-responsive (congenital) sideroblastic anaemia may be due to the presence of an abnormal ALA-synthetase apoenzyme which requires excessive amounts of the coenzyme, pyridoxal-phosphate, to achieve normal activity; other abnormalities of haem synthesis may also be present." @default.
- W1986018588 created "2016-06-24" @default.
- W1986018588 creator A5031429272 @default.
- W1986018588 creator A5061926072 @default.
- W1986018588 date "1979-05-01" @default.
- W1986018588 modified "2023-10-16" @default.
- W1986018588 title "Haem Synthesis in Sideroblastic Anaemia" @default.
- W1986018588 cites W1497505325 @default.
- W1986018588 cites W1969809945 @default.
- W1986018588 cites W1991719441 @default.
- W1986018588 cites W2007062440 @default.
- W1986018588 cites W2015613619 @default.
- W1986018588 cites W2024592423 @default.
- W1986018588 cites W2026468757 @default.
- W1986018588 cites W2036580971 @default.
- W1986018588 cites W2065623647 @default.
- W1986018588 cites W2132499895 @default.
- W1986018588 cites W2147104516 @default.
- W1986018588 cites W2155588768 @default.
- W1986018588 cites W22357923 @default.
- W1986018588 cites W2270510886 @default.
- W1986018588 cites W2320934986 @default.
- W1986018588 cites W2415554736 @default.
- W1986018588 cites W255566741 @default.
- W1986018588 cites W95338710 @default.
- W1986018588 doi "https://doi.org/10.1111/j.1365-2141.1979.tb03699.x" @default.
- W1986018588 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/465361" @default.
- W1986018588 hasPublicationYear "1979" @default.
- W1986018588 type Work @default.
- W1986018588 sameAs 1986018588 @default.
- W1986018588 citedByCount "51" @default.
- W1986018588 countsByYear W19860185882014 @default.
- W1986018588 countsByYear W19860185882017 @default.
- W1986018588 countsByYear W19860185882020 @default.
- W1986018588 countsByYear W19860185882022 @default.
- W1986018588 crossrefType "journal-article" @default.
- W1986018588 hasAuthorship W1986018588A5031429272 @default.
- W1986018588 hasAuthorship W1986018588A5061926072 @default.
- W1986018588 hasConcept C126322002 @default.
- W1986018588 hasConcept C134018914 @default.
- W1986018588 hasConcept C142724271 @default.
- W1986018588 hasConcept C181199279 @default.
- W1986018588 hasConcept C185592680 @default.
- W1986018588 hasConcept C197957613 @default.
- W1986018588 hasConcept C2776514930 @default.
- W1986018588 hasConcept C2776834122 @default.
- W1986018588 hasConcept C2776967927 @default.
- W1986018588 hasConcept C2780007613 @default.
- W1986018588 hasConcept C2780859938 @default.
- W1986018588 hasConcept C2781069245 @default.
- W1986018588 hasConcept C2909176517 @default.
- W1986018588 hasConcept C55493867 @default.
- W1986018588 hasConcept C71924100 @default.
- W1986018588 hasConceptScore W1986018588C126322002 @default.
- W1986018588 hasConceptScore W1986018588C134018914 @default.
- W1986018588 hasConceptScore W1986018588C142724271 @default.
- W1986018588 hasConceptScore W1986018588C181199279 @default.
- W1986018588 hasConceptScore W1986018588C185592680 @default.
- W1986018588 hasConceptScore W1986018588C197957613 @default.
- W1986018588 hasConceptScore W1986018588C2776514930 @default.
- W1986018588 hasConceptScore W1986018588C2776834122 @default.
- W1986018588 hasConceptScore W1986018588C2776967927 @default.
- W1986018588 hasConceptScore W1986018588C2780007613 @default.
- W1986018588 hasConceptScore W1986018588C2780859938 @default.
- W1986018588 hasConceptScore W1986018588C2781069245 @default.
- W1986018588 hasConceptScore W1986018588C2909176517 @default.
- W1986018588 hasConceptScore W1986018588C55493867 @default.
- W1986018588 hasConceptScore W1986018588C71924100 @default.
- W1986018588 hasIssue "1" @default.
- W1986018588 hasLocation W19860185881 @default.
- W1986018588 hasLocation W19860185882 @default.
- W1986018588 hasOpenAccess W1986018588 @default.
- W1986018588 hasPrimaryLocation W19860185881 @default.
- W1986018588 hasRelatedWork W145542825 @default.
- W1986018588 hasRelatedWork W1467865937 @default.
- W1986018588 hasRelatedWork W1529381705 @default.
- W1986018588 hasRelatedWork W1928077593 @default.
- W1986018588 hasRelatedWork W1970461912 @default.
- W1986018588 hasRelatedWork W1971214444 @default.
- W1986018588 hasRelatedWork W2071806336 @default.
- W1986018588 hasRelatedWork W2318556522 @default.
- W1986018588 hasRelatedWork W2334839981 @default.
- W1986018588 hasRelatedWork W2748952813 @default.
- W1986018588 hasVolume "42" @default.
- W1986018588 isParatext "false" @default.
- W1986018588 isRetracted "false" @default.
- W1986018588 magId "1986018588" @default.
- W1986018588 workType "article" @default.