In 13 subjects with ALS, we studied the safety and pharmacokinetic properti
es of Procysteine, a cysteine prodrug that increases levels of intracellula
r glutathione. We found that oral administration of Procysteine was safe. P
rocysteine enters CSF after both IV and oral dosing and accumulates to sign
ificant levels in CSF. We also observed that CSF levels of glutathione fall
dramatically with aging.