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Plasma BCAAs and alloIsoleucine of relatives.
| Plasma BCAAs (Reference Range Values) |
LEU (77–195 μmol/L) |
ILE (38–99 μmol/L) |
VAL (130–335 μmol/L) |
ALLOILE (<2.0 μmol/L) |
|---|---|---|---|---|
| Patient | 428 | 207 | 418 | 17.0 |
| Father | 384 | 156 | 403 | 14.0 |
| Paternal grandfather | 234 | 111 | 315 | 3.6 |
Abbreviations: BCAAs, branched-chain amino acids; LEU, leucine; ILE, isoleucine; VAL, valine; ALLOILE, alloisoleucine. In bold pathological values.
