Abstract
Myopathy in glycogen storage disease type II (GSD-II) is slowly progressive. Five subjects with the late-onset form of GSD-II (age range, 15 to 47 years) and seven healthy control subjects (age range, 28 to 55 years) were studied. Following alanine supplementation, resting energy expenditure decreased in patients with GSD-II (p < 0.05) compared to values seen in control subjects. Leucine flux decreased (p < 0.004), as did leucine oxidation, to levels lower than those observed in control subjects (p < 0.001). L-Alanine reduces protein turnover and catabolism in GSD-II.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 710-712 |
| Number of pages | 3 |
| Journal | Neurology |
| Volume | 55 |
| Issue number | 5 |
| DOIs | |
| State | Published - 2000 |
ASJC Scopus subject areas
- Clinical Neurology