Document Detail

delta-Aminolevulinic acid synthetase in erythroblasts of patients with pyridoxine-responsive anemia. Hypercatabolism caused by the increased susceptibility to the controlling protease.
MedLine Citation:
PMID:  500806     Owner:  NLM     Status:  MEDLINE    
Properties of delta-aminolevulinic acid synthetase in erythroblasts of patients with pyridoxine-responsive anemia were investigated with special reference to the protease in mitochondria of erythroblasts. delta-Aminolevulinic acid synthetase activity in erythroblasts of patients with this disease before treatment was extremely decreased, whereas it gradually increased in parallel with the improvement of anemia by the therapy with pyridoxal phosphate. The amount of apo-delta-aminolevulinic acid synthetase in erythroblasts before treatment was also extremely diminished. Apparent affinity to pyridoxal phosphate of the apo-delta-aminolevulinic acid synthetase obtained from erythroblasts of the patients was almost the same as that of normal controls. The activity of a new protease which is considered to be engaged in the regulation of delta-aminolevulinic acid synthetase levels in mitochondria of erythroblasts was shown to be in normal range in erythroblasts of the patients. On the other hand, apo-delta-aminolevulinic acid synthetase obtained from the patients was extremely sensitive to the protease. These results indicate that disturbance of heme synthesis characteristic to pyridoxine-responsive anemia could be ascribed to the hypercatabolism of delta-aminolevulinic acid synthetase caused by the increased susceptibility to the controlling protease in erythroblasts.
Y Aoki; S Muranaka; K Nakabayashi; Y Ueda
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  The Journal of clinical investigation     Volume:  64     ISSN:  0021-9738     ISO Abbreviation:  J. Clin. Invest.     Publication Date:  1979 Nov 
Date Detail:
Created Date:  1980-01-28     Completed Date:  1980-01-28     Revised Date:  2009-11-18    
Medline Journal Info:
Nlm Unique ID:  7802877     Medline TA:  J Clin Invest     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  1196-203     Citation Subset:  AIM; IM    
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MeSH Terms
5-Aminolevulinate Synthetase / metabolism*
Anemia, Hypochromic / drug therapy,  enzymology*
Anemia, Sideroblastic / drug therapy,  enzymology*
Bone Marrow / enzymology*,  ultrastructure
Bone Marrow Cells
Endopeptidases / metabolism
Enzyme Inhibitors / metabolism
Mitochondria / enzymology*
Pyridoxal Phosphate / therapeutic use
Vitamin B 6 Deficiency / drug therapy,  metabolism
Reg. No./Substance:
0/Enzyme Inhibitors; 54-47-7/Pyridoxal Phosphate; EC Synthetase; EC 3.4.-/Endopeptidases

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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