Free amino acid levels in amyotrophic lateral sclerosis.

Abstract:

:To evaluate reports of abnormal levels of free amino acids (AA) in patients with amyotrophic lateral sclerosis (ALS), we studied serum, cerebrospinal fluid, and urine AA in 12 patients with ALS and 12 controls matched for age, sex, and severity of disability. ALS patients had statistically significant elevations in serum levels of tyrosine, total aromatic AA, and total basic AA. ALS patients also had statistically significant elevations in cerebrospinal fluid of total basic AA, lysine, essential AA, and leucine. The severity of ALS correlated inversely with acidic AA (glutamate and aspartate) and O-phosphoserine in cerebrospinal fluid. Activity of ALS correlated directly with serum aspartate and cerebrospinal fluid alanine. We conclude that subtle abnormalities of AA levels are present in ALS and that these are not due to age, sex, or disability. The pattern of distribution of AA levels differs from that in hepatic or renal disease and suggests defective membrane transport or poor cellular utilization of basic and essential AA in the central nervous system.

journal_name

Ann Neurol

journal_title

Annals of neurology

authors

Patten BM,Harati Y,Acosta L,Jung SS,Felmus MT

doi

10.1002/ana.410030405

subject

Has Abstract

pub_date

1978-04-01 00:00:00

pages

305-9

issue

4

eissn

0364-5134

issn

1531-8249

journal_volume

3

pub_type

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