SMN1 and proximal spinal muscular atrophy: Our metabolomic study in SMNΔ7 mice unveiled that SMN deficiency induces a prominent ArAA and BCAA dysmetabolism, disclosing the risk of a deleterious hepatic depletion of these vital molecules in SMA, especially in the most severe forms, in which a boost in amino acid consumption for fueling defective glucose metabolism is required11,12,15,16,22,71.