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Branched‐chain ketoacids reduce muscle protein degradation in duchenne muscular dystrophy
Author(s) -
Stewart Peter M.,
Walser Mackenzie,
Drachman Daniel B.
Publication year - 1982
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.880050304
Subject(s) - duchenne muscular dystrophy , excretion , muscular dystrophy , medicine , creatinine , endocrinology , muscle protein , ornithine , alpha (finance) , chemistry , biochemistry , skeletal muscle , amino acid , surgery , arginine , construct validity , patient satisfaction
In order to determine whether branched‐chain ketoacids can reduce the excessive rate of muscle protein degradation that characterizes Duchenne muscular dystrophy, nine boys affected with the disease were studied in a metabolic ward while receiving meat‐free diets. After a three‐day equilibration period, excretion rates of 3‐methylhistidine and creatinine were measured in two consecutive four‐day periods. In the second period, a supplement containing a mixture of ornithine α‐ketoisocaproate, α‐ketoisovalerate, and α‐keto‐β‐methylvalerate in a proportion of 4 : 1 : 1 was administered orally at a dosage of 0.45 gm/kg/day. During treatment with the ketoacids, 3‐methylhistidine excretion fell by a small (mean: 14%) but highly significant ( P < 0.01) extent, whether expressed in absolute terms or in relation to creatinine excretion. No adverse effects were noted. We conclude that this mixture of ketoacids acutely reduces muscle protein degradation in patients with Duchenne muscular dystrophy.