X-linked creatine deficiency syndrome: a novel mutation in creatine transporter gene SLC6A8.

Bizzi, Alberto; Bugiani, Marianna; Salomons, Gajja S; Hunneman, Donald H; Moroni, Isabella; Estienne, Margherita; Danesi, Ugo; Jakobs, Cornelis et al. · Ann Neurol · 2002

case_report · Level V

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Abstract

Among creatine deficiency syndromes, an X-linked condition related to a defective creatine transport into the central nervous system has been described recently. Hallmarks of the disease are the absence of a creatine signal at brain spectroscopy, increased creatine levels in blood and urine, ineffectiveness of oral supplementation, and a mutation in the SLC6A8 (Online Mendelian Inheritance in Man [OMIM] 300036) creatine transporter gene. We report on a patient in whom a novel mutation (1221-1223delTTC) was identified.

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