Suppression of granulopoietic progenitor cell proliferation by metabolites of the branched-chain amino acids | Health & Environmental Research Online (HERO)

Health & Environmental Research Online (HERO)

Print Feedback Export to File

This record has one attached file:

Citation
Tags

HERO ID

4923763

Reference Type

Journal Article

Title

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

Author(s)

Hutchinson, RJ; Bunnell, K; Thoene, JG

Year

1985

Is Peer Reviewed?

Yes

Journal

Journal of Pediatrics
ISSN: 0022-3476
EISSN: 1097-6833

Volume

106

Issue

Page Numbers

62-65

Language

English

PMID

3965682

DOI

10.1016/S0022-3476(85)80466-2

Web of Science Id

A1985AAH1500012

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.