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Arylsulfatase A pseudodeficiency in healthy Brazilian individuals.
Pedron, C G; Gaspar, P A; Giugliani, R; Pereira, M L.
Afiliación
  • Pedron CG; Servico de Genética Médica, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brasil.
Braz J Med Biol Res ; 32(8): 941-5, 1999 Aug.
Article en En | MEDLINE | ID: mdl-10454754
Molecular alterations associated with arylsulfatase A pseudodeficiency (ASA-PD) were characterized by PCR and restriction endonuclease analysis in a sample of healthy individuals from Brazil. ASA activity was also assayed in all subjects. Two individuals homozygous for the N350S and 1524+95A<--G mutations were detected, corresponding to a frequency of 1.17% (4 of 324 alleles). The individual frequency of the N350S mutation was 20.7% (71 of 342 alleles) and 7.9% (27 of 342 alleles) for the 1524+95A<--G mutation. The frequency of the ASA-PD allele in our population was estimated to be 7.9%. This is the first report of ASA-PD allele frequency in a South American population. In addition, the methods used are effective and suitable for application in countries with limited resources. All patients with low ASA activity should be screened for ASA-PD as part of the diagnostic protocol for metachromatic leukodystrophy.
Asunto(s)
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Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Cerebrósido Sulfatasa / Alelos / Leucodistrofia Metacromática Tipo de estudio: Guideline Límite: Female / Humans / Male País/Región como asunto: America do sul / Brasil Idioma: En Revista: Braz J Med Biol Res Año: 1999 Tipo del documento: Article País de afiliación: Brasil Pais de publicación: Brasil
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Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Cerebrósido Sulfatasa / Alelos / Leucodistrofia Metacromática Tipo de estudio: Guideline Límite: Female / Humans / Male País/Región como asunto: America do sul / Brasil Idioma: En Revista: Braz J Med Biol Res Año: 1999 Tipo del documento: Article País de afiliación: Brasil Pais de publicación: Brasil