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L-Arginine:glycine amidinotransferase deficiency

ORPHA:35704· ICD-10 E72.8

Definition

L-Arginine:glycine amidinotransferase (AGAT) deficiency is a very rare type of creatine deficiency sydrome characterized by global developmental delay, intellectual disability, and myopathy.

Prevalence
<1 / 1 000 000
Inheritance
Autosomal recessive
Age of onset
Infancy