Abstract
We read with interest the paper by Bianchi et al,[1][1] in which the authors report on the treatment monitoring of 2 patients with guanidinoacetate methyltransferase (GAMT) deficiency and 3 patients with an arginine:glycine amidinotransferase defect (AGAT-d). Repetitive MR measurements of these
Highlights
Bianchi et al[1] studied the levels of brain creatine (Cr) and phosphocreatine in 1 patient with guanidinoacetate methyltransferase (GAMT) deficiency using 31P- and 1H-MR spectroscopy before and after therapy
According to Bianchi et al,[1] the supposed elevation of adenosine triphosphate (ATP) is based on the 31P-MR spectrum of GAMT; in patient 1, in particular, the signal intensity for -ATP is increased
If ATP were elevated, why are not all 3 signals elevated, since they belong to the same molecule? the other ATP signals (␣-ATP and ␥-ATP) represent other phosphorus compounds such as adenosine diphosphate (ADP), these are present at much lower concentrations (ϳM range) than ATP
Summary
Bianchi et al[1] studied the levels of brain creatine (Cr) and phosphocreatine in 1 patient with GAMT deficiency using 31P- and 1H-MR spectroscopy (phosphorous and proton MR spectroscopy) before and after therapy. According to Bianchi et al,[1] the supposed elevation of ATP is based on the 31P-MR spectrum of GAMT; in patient 1, in particular, the signal intensity for -ATP (at 16.5 ppm) is increased. ATP has 3 phosphate groups, all giving a distinct signal intensity at different positions on the 31P-MR spectrum.
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