The selective removal of red blood cells from bone marrow cell suspensions

To examine the role of 5-aminolevulinate synthase (ALAS) in the pathogenesis of sideroblastic anemias, levels of mRNAs for erythroid and housekeeping ALAS isozymes were examined, and total ALAS activity was assessed in bone marrow cells. In two patients with X-linked sideroblastic anemia the levels of mRNA for erythroid ALAS as well as for alpha and beta globin appear to be decreased while levels of mRNA for glycophorin A in both patients were the same as in normal individuals. However, amounts of housekeeping ALAS mRNA were increased two- to threefold in these patients. Total ALAS activity was also increased two- or threefold, perhaps reflecting increased transcription of the housekeeping gene in response to diminished cellular heme in erythroid cells and/or enhanced translation of the erythroid isoform in response to iron accumulation. In a third patient with X-linked sideroblastic anemia ALAS activity was low but increased to twice the normal value after pyridoxine administration, suggesting a structural defect of the enzyme. In a fourth patient, with isolated congenital, pyridoxine-responsive sideroblastic anemia, the erythroid ALAS mRNA was normal and a low enzyme activity was strikingly enhanced by pyridoxal-phosphate albeit to subnormal levels. In idiopathic acquired sideroblastic anemia, ALAS mRNA for both isozymes was normal and enzyme activity was slightly elevated. These observations thus reflect heterogeneous aberrations of erythroid heme synthesis in the various types of sideroblastic anemia and suggest that defects involving erythroid ALAS underlie at least some of them.

show abstract

5‐Aminolevulinate synthase in sideroblastic anemias: mRNA and enzyme activity levels in bone marrow cells

Bottomley

Healy

Brandenburg

et al. 1992

American J Hematol

View full text Add to dashboard Cite

show abstract

Protection of bone marrow progenitor cells by superoxide dismutase

Petkau

1988

Mol Cell Biochem

View full text Add to dashboard Cite

Intravenously administered cuprozinc-superoxide dismutase in X-irradiated mice hastens the recovery of peripheral blood cells. This effect is consistent with protection of the pluripotent stem cells by the enzyme. Amongst the bone marrow cells committed to differentiation along the myeloid pathway, there exists in mice a subpopulation of macrophage progenitor cells that is inactivated by superoxide radicals, generated photochemically or by X-rays. This cell killing effect is inhibited by superoxide dismutase, in part because it acts intracellularly. Human bone marrow also contain a superoxide-sensitive subpopulation of myeloid progenitor cells that is protected by superoxide dismutase but not by catalase. As well, human myeloid progenitor cells contain a subpopulation with enhanced sensitivity to X-rays in vitro. Treatment of these cells with exogenous superoxide dismutase reduces the sensitivity to X-rays by a factor of 2.

show abstract

Endogenous production of prothymocyte differentiating activity by phytohemagglutinin-stimulated T-cell-depleted human marrow

Ahmad¹,

Lecron²,

Descombe³

et al. 1986

Cellular Immunology

View full text Add to dashboard Cite

scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.

Contact Info

hi@scite.ai

10624 S. Eastern Ave., Ste. A-614

Henderson, NV 89052, USA

Blog Terms and Conditions API Terms Privacy Policy Contact Cookie Preferences Do Not Sell or Share My Personal Information

Made with 💙 for researchers

Part of the Research Solutions Family.

The selective removal of red blood cells from bone marrow cell suspensions

Cited by 11 publications

References 6 publications

5‐Aminolevulinate synthase in sideroblastic anemias: mRNA and enzyme activity levels in bone marrow cells

5‐Aminolevulinate synthase in sideroblastic anemias: mRNA and enzyme activity levels in bone marrow cells

Protection of bone marrow progenitor cells by superoxide dismutase

Endogenous production of prothymocyte differentiating activity by phytohemagglutinin-stimulated T-cell-depleted human marrow

Contact Info

Product

Resources

About