The Neurotoxicity of Valine Deficiency in Rats

Abstract

When valine, an essential amino acid, was withdrawn from the diet of weanling rats, the animals rapidly developed a unique pattern of neurological symptoms characterized by head retraction, staggering and aimless circling. At necropsy degenerative changes were most prominent in the neurons of the red nuclei, brain stem structures which modulate motor function. To explore the pathogenesis of the neurotoxicity associated with valine deficiency, we fed rats purified diets deficient in valine alone or in valine plus other branched chain and neutral amino acids, and we examined brain tissues by light microscopy. Motor disfunction and red nuclei damage occurred only in rats fed diets lacking valine alone and not in rats fed diets lacking all three branched chain amino acids. These results suggest that the neurotoxicity of valine deficiency results from amino acid imbalance rather than from lack of dietary valine per se.

Metabolite profiling for the identification of altered metabolic pathways in Alzheimer's disease

“…of the GSH/GSSG ratio, diminished ␥-glutamylcysteine synthetase activity in de novo glutathione synthesis and increased activity ington's disease [15]. Valine deficiency is related to neurological 412 defects in rats [64], but its relation with AD is not clear. comprehensive metabolomic profiles of serum samples, useful for the discrimination of AD patients from healthy controls.…”

Branched-chain amino acid depletion conditions bone marrow for hematopoietic stem cell transplantation avoiding amino acid imbalance-associated toxicity

“…Valine is a one of three branched-chain amino acids (BCAAs) in addition to isoleucine and leucine. BCAA imbalance has been suggested to cause cellular toxicity, including neurotoxicity [8] . Stimulated by these reports, we investigated the sensitivity of HSCs to BCAA imbalance using ex vivo HSC expansion cultures.…”

Valine Requirement for Reproduction in Swine

“…Based upon these findings (Rose and Eppstein, 1939), the authors classified valine as an indispensable dietary component. The neurotoxicity of valine deficiency also was demonstrated in a recent study (Cusick et al_., 1978).…”