Heme biosynthesis in intermittent acute prophyria: decreased hepatic conversion of porphobilinogen to porphyrins and increased delta aminolevulinic acid synthetase activity.
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Heme synthetase deficiency in human protoporphyria. Demonstration of the defect in liver and cultured skin fibroblastsAn inherited enzymatic defect in porphyria cutanea tarda: decreased uroporphyrinogen decarboxylase activityHepatic heme metabolism and its controlStudies in porphyria. IV. Expression of the gene defect of acute intermittent porphyria in cultured human skin fibroblasts and amniotic cells: prenatal diagnosis of the porphyric traitQuantitative studies of the delivery of hepatic-synthesized bilirubin to plasma utilizing -aminolevulinic acid-4- 14 C and bilirubin- 3 H in manThe effects of drugs on the activities of 5-aminolaevulinate synthetase and other enzymes in the pathway of haem biosynthesisFamily evaluations in acute intermittent porphyria using red cell uroporphyrinogen I synthetase.Screening for latent acute intermittent porphyria: the value of measuring both leucocyte delta-aminolaevulinic acid synthase and erythrocyte uroporphyrinogen-1-synthase activitiesAAV8-mediated gene therapy prevents induced biochemical attacks of acute intermittent porphyria and improves neuromotor function.Motor neuropathy in porphobilinogen deaminase-deficient mice imitates the peripheral neuropathy of human acute porphyria.Theodore Woodward Award. Pathogenesis of biochemical abnormalities in protoporphyria.The clinical biochemistry of the human hepatocutaneous porphyrias in the light of recent studies of newly identified intermediates and porphyrin derivatives.Comparative aspects of porphyria in man and animals.Inosinic acid dehydrogenase activity in the Lesch-Nyhan syndrome.Molecular variation in relation to purine metabolismGenetic heterogeneity in acute intermittent porphyria: characterisation and frequency of porphobilinogen deaminase mutations in FinlandAcute intermittent porphyria: characterization of a novel mutation in the structural gene for porphobilinogen deaminase. Demonstration of noncatalytic enzyme intermediates stabilized by bound substratePostulated deficiency of hepatic heme and repair by hematin infusions in the "inducible" hepatic porphyrias.Human phosphoribosylpyrophosphate synthetase: increased enzyme specific activity in a family with gout and excessive purine synthesis.Decreased red cell uroporphyrinogen I synthetase activity in intermittent acute porphyriaThe role of iron in the pathogenesis of porphyria cutanea tarda. An in vitro model.Induction of a deficiency of steroid delta 4-5 alpha-reductase activity in liver by a porphyrinogenic drugStudies in porphyria. I. A defect in the reductive transformation of natural steroid hormones in the hereditary liver disease, acute intermittent porphyria.Regional gene assignment of human porphobilinogen deaminase and esterase A4 to chromosome 11q23 leads to 11qter.Rare structural variants of human and murine uroporphyrinogen I synthase.The porphyrias: a reviewCharacterization of the porphobilinogen deaminase deficiency in acute intermittent porphyria. Immunologic evidence for heterogeneity of the genetic defectStudies in porphyria. VII. Induction of uroporphyrinogen-I synthase and expression of the gene defect of acute intermittent porphyria in mitogen-stimulated human lymphocytesA microassay for uroporphyrinogen I synthase, one of three abnormal enzyme activities in acute intermittent porphyria, and its application to the study of the genetics of this diseasePresynaptic neuromuscular inhibition by porphobilinogen and porphobilin.Repression of the overproduction of porphyrin precursors in acute intermittent porphyria by intravenous infusions of hematin.Liver in disorders of porphyrin metabolism.Quantitation of 3-ethyl-5-hydroxy-4,5-dimethyl-delta 3-pyrrolin-2-one in the urine of patients with acute intermittent porphyria.The control of haemoglobin synthesis.Recent advances in porphyrin metabolism.The biochemical diagnosis of the porphyrias.Simultaneous determination of hydroxymethylbilane synthase and uroporphyrinogen III synthase in erythrocytes by high-performance liquid chromatography.Factors determining the sequence of oxidative decarboxylation of the 2- and 4-propionate substituents of coproporphyrinogen III by coproporphyrinogen oxidase in rat liver.Conversion of 5-aminolaevulinate into haem by liver homogenates. Comparison of rat and chick embryo.Marked geographic aggregation of acute intermittent porphyria families carrying mutation Q180X in Venezuelan populations, with description of further mutations.
P2860
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P2860
Heme biosynthesis in intermittent acute prophyria: decreased hepatic conversion of porphobilinogen to porphyrins and increased delta aminolevulinic acid synthetase activity.
description
1970 nî lūn-bûn
@nan
1970 թուականի Նոյեմբերին հրատարակուած գիտական յօդուած
@hyw
1970 թվականի նոյեմբերին հրատարակված գիտական հոդված
@hy
1970年の論文
@ja
1970年論文
@yue
1970年論文
@zh-hant
1970年論文
@zh-hk
1970年論文
@zh-mo
1970年論文
@zh-tw
1970年论文
@wuu
name
Heme biosynthesis in intermitt ...... inic acid synthetase activity.
@ast
Heme biosynthesis in intermitt ...... inic acid synthetase activity.
@en
type
label
Heme biosynthesis in intermitt ...... inic acid synthetase activity.
@ast
Heme biosynthesis in intermitt ...... inic acid synthetase activity.
@en
prefLabel
Heme biosynthesis in intermitt ...... inic acid synthetase activity.
@ast
Heme biosynthesis in intermitt ...... inic acid synthetase activity.
@en
P2093
P2860
P356
P1476
Heme biosynthesis in intermitt ...... inic acid synthetase activity.
@en
P2093
P2860
P304
P356
10.1073/PNAS.67.3.1315
P407
P577
1970-11-01T00:00:00Z