delta Aminolevulinate synthase
[Recent progress in iron metabolism and iron-related anemia].
Sendai, Japan. In Rinsho Byori, 2010
The most common inherited sideroblastic anemia is X-linked sideroblastic anemia (XLSA) caused by mutations of the erythroid-specific delta-aminolevulinate synthase gene (ALAS2), which is the first enzyme involved in heme biosynthesis in erythroid cells.
Light pulse induces ALA-S gene expression in the rat Harderian gland.
Paris, France. In J Physiol Pharmacol, 2010
We have previously shown that continuous light exposure abolished the day/night variations of the delta-aminolevulinate synthase (ALA-S; the rate-limiting enzyme for porphyrin biosynthesis) gene expression observed under standard light: dark cycles (LD 12:12) in the rat HGs.
PBRL, a putative peripheral benzodiazepine receptor, in primitive erythropoiesis.
Kōbe, Japan. In Gene Expr Patterns, 2009
Five of them, delta-aminolevulinate synthase, delta-aminolevulinic acid dehydrogenase, porphobilinogen deaminase, coproporphyrinogen decarboxylase and ferrochelatase, show stage-specific increase in gene expression correlated with primitive hematopoiesis, but not with primitive erythrocyte differentiation.
B6-responsive disorders: a model of vitamin dependency.
London, United Kingdom. In J Inherit Metab Dis, 2006
The last show a very variable degree of pyridoxine responsiveness, from 90% in X-linked sideroblastic anaemia (delta-aminolevulinate synthase deficiency) through 50% in homocystinuria (cystathionine beta-synthase deficiency) to 5% in ornithinaemia with gyrate atrophy (ornithine delta-aminotransferase deficiency).
Multiple mechanisms for hereditary sideroblastic anemia.
Sendai, Japan. In Cell Mol Biol Incl Cyto Enzymol, 2002
It has been shown that a deficiency of the erythroid-specific delta-aminolevulinate synthase (ALAS-E) activity is responsible for pyridoxine-responsive HSA in many patients, however, the pathogenesis of other types of HSA remains still unknown.
[Heme metabolism and oxidative stress].
Kharkiv, Ukraine. In Ukr Biokhim Zh (1999), 2001
The main attention is focused to the prooxidant action of heme, the interaction of heme transport and lipid exchange, and to the heme metabolism key enzymes (delta-aminolevulinate synthase and heme oxygenase), serum heme-binding protein hemopexin and intracellular heme-binding proteins participating in metabolism adaptation under the action of factors, which cause oxidative stress.