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[Mutation P28T in gene GK1 as the cause of a familial galactokinase deficiency]. / La mutación P28T del gen GK1 como causa de una deficiencia familiar de galactoquinasa.
Girós, M; Bóveda, M D; Vázquez de la Cruz, A; Lázaro, P; Gata, A; Solar Boga, A; Briones, P.
Afiliación
  • Girós M; Institut de Bioquímica Clínica, Corporació Sanitaria, Barcelona, España. mgiros@clinic.ub.es
Arch Soc Esp Oftalmol ; 78(2): 111-4, 2003 Feb.
Article en Es | MEDLINE | ID: mdl-12647253
OBJECTIVE/METHOD: To alert about galactokinase deficiency (GK) as a possible cause of infantile cataracts, and even presenile cataracts in heterozygous carriers. Diagnosis by enzyme and galactitol determination would lead to the introduction of a galactose-free diet which completely prevents the damage. RESULT/CONCLUSIONS: We report on a highly consanguineous Spanish family of gypsy ethnia, with three females of different sibships affected by GK deficiency. The deficiency was due to their homozygosis for mutation P28T in gene GK1. P28T mutation in european Romani gypsies, is also present in Spanish gypsies. It is important to bear in mind that GK deficiency may be an important cause of blindness in that endogamous group.
Asunto(s)
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Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Romaní / Galactoquinasa / Mutación Tipo de estudio: Etiology_studies Límite: Female / Humans / Infant / Male Idioma: Es Revista: Arch Soc Esp Oftalmol Año: 2003 Tipo del documento: Article Pais de publicación: España
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Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Romaní / Galactoquinasa / Mutación Tipo de estudio: Etiology_studies Límite: Female / Humans / Infant / Male Idioma: Es Revista: Arch Soc Esp Oftalmol Año: 2003 Tipo del documento: Article Pais de publicación: España