Suppression of granulopoietic progenitor cell proliferation by metabolites of the branched-chain amino acids

Hutchinson, RJ; Bunnell, K; Thoene, JG

HERO ID

4923763

Reference Type

Journal Article

Year

1985

Language

English

PMID

3965682

HERO ID 4923763
In Press No
Year 1985
Title Suppression of granulopoietic progenitor cell proliferation by metabolites of the branched-chain amino acids
Authors Hutchinson, RJ; Bunnell, K; Thoene, JG
Journal Journal of Pediatrics
Volume 106
Issue 1
Page Numbers 62-65
Abstract The effects of branched-chain amino acid metabolites on granulocyte-macrophage progenitor cell proliferation in marrow culture are reported. Isovalerate and propionate profoundly suppress granulopoiesis at both 3.2 and 6.4 mM concentrations, whereas methylmalonate and other metabolites suppress to a lesser degree. The parent branched-chain amino acids leucine, isoleucine, and valine do not suppress in vitro granulopoiesis at similar concentrations. Because the concentrations of the organic acids tested fall within the pathophysiologic ranges observed in patients with isovaleric, propionic, and methylmalonic acidemias, we suggest that elevated in vivo levels of isovalerate, propionate, and to a lesser degree methylmalonate are responsible for the neutropenia observed in these disorders.
Doi 10.1016/S0022-3476(85)80466-2
Pmid 3965682
Wosid A1985AAH1500012
Is Certified Translation No
Dupe Override No
Is Public Yes
Language Text English