Saturday Sep 20, 2014 (foodconsumer.org) -- A new study suggests that L-leucine supplementation may help Pompe disease, which can lead to muscle dysfunction, and strengthen muscle function in the patients.

Acid-α-glucosidase (GAA) deficiency found in patients with Pompe disease is linked to decreased mTORC1 activation.  The current study led by Hailong Zong at Albert Einstein College of Medicine and colleagues demonstrated that supplementation with cell permeable leucine analog L-Leucyl-L-leucine methyl ester restored mTORC1 activation.

In vivo, a combination of insulin and L-leucine supplementation normalized mTORC1 activation in mice with Pompe disease, which otherwise experienced reduced basal and leucine stimulated mTORC1 activation in skeletal muscle.

Additionally, the study revealed chronic L-leucine supplementation restored basal and leucine stimulated mTORC1 activation and partially protected mice with Pompe disease from suffering kyphosis and muscle loss.

Mice with Pompe disease supplemented with L-leucine reduced muscle breakdown, increased glycogen accumulation, and experienced increased spontaneous activity and running capacity.

Pompe disease can lead to the development of skeletal muscle dysfunction and leucine supplementation can help at least partially protect against Pompe disease.  

L-Leucine as branched-chain amino acid (BCAA) along with other branched chain amino acids isoleucine and valine account for 35% protein mass in muscle tissue.  Body builders take branched chain amino acids to build muscle mass and older people take these amino acids to reduce age-related muscle loss.  Older people are expected to lose 1% of muscle per year.

Note that L-leucine is natural while DL-leucine is synthetic.  (David Liu, PHD)

Shemesh A, Wang Y, Yang Y, Yang GS, Johnson DE, Backer JM, Pessin  JE, Zong H.   Suppression of mTOR1 Activation in Acid- α -Glucosidase Deficient Cells and Mice is Ameliorated by Leucine Supplementation.  Am J Physiol Regul Integr Comp Physiol. 2014 Sep 17.