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Alpha-l-iduronidase and arylsulfatase B in dried blood spots on filter paper: Biochemical parameters and time stability.
Breier, Ana Carolina; Cé, Jaqueline; Mezzalira, Jamila; Daitx, Vanessa V; Moraes, Vitoria C; Goldim, Mariana P S; Coelho, Janice C.
Afiliación
  • Breier AC; Postgraduate Program, Biochemistry Department, Lysosomal Storage Diseases Laboratory, Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS, Brazil. Electronic address: anacarolina.breier@gmail.com.
  • Cé J; Postgraduate Program, Biochemistry Department, Lysosomal Storage Diseases Laboratory, Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS, Brazil.
  • Mezzalira J; Postgraduate Program, Biochemistry Department, Lysosomal Storage Diseases Laboratory, Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS, Brazil.
  • Daitx VV; Postgraduate Program, Biochemistry Department, Lysosomal Storage Diseases Laboratory, Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS, Brazil.
  • Moraes VC; Postgraduate Program, Biochemistry Department, Lysosomal Storage Diseases Laboratory, Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS, Brazil.
  • Goldim MPS; Postgraduate Program, Biochemistry Department, Lysosomal Storage Diseases Laboratory, Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS, Brazil.
  • Coelho JC; Postgraduate Program, Biochemistry Department, Lysosomal Storage Diseases Laboratory, Universidade Federal do Rio Grande do Sul (UFRGS), Porto Alegre, RS, Brazil.
Clin Biochem ; 50(7-8): 431-435, 2017 May.
Article en En | MEDLINE | ID: mdl-28088454
BACKGROUND: The goal of this study was to assess the biochemical parameters of the enzymes α-l-iduronidase (IDUA) and arylsulfatase B (ASB), which are deficient in mucopolysaccharidosis (MPS) I and VI, respectively, in dried blood spot (DBS) samples impregnated on filter paper. METHODS AND RESULTS: The optimal pH, Km, and Vmax of IDUA and ASB in DBS are hereby presented. After these analyses, the reference values for the activities of these enzymes in DBS with cutoff of 3.65nmol/h/mL for IDUA and 6.80nmol/h/mL for ASB were established. The research also showed that the stability (21days) of the IDUA activity is lower than ASB, which maintained its enzymatic activity stable up until 60days of analysis, after impregnating the filter paper with blood. CONCLUSION: Currently, DBS ensures important advantages in handling storage and transportation of samples with respect to neonatal screening programs. This study contributes to characterizing and differentiating the biochemistry of deficient enzymes in MPSs I and VI of DBS samples.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Mucopolisacaridosis I / Mucopolisacaridosis IV / N-Acetilgalactosamina-4-Sulfatasa / Pruebas con Sangre Seca / Iduronidasa Límite: Female / Humans / Male Idioma: En Revista: Clin Biochem Año: 2017 Tipo del documento: Article Pais de publicación: Estados Unidos
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Mucopolisacaridosis I / Mucopolisacaridosis IV / N-Acetilgalactosamina-4-Sulfatasa / Pruebas con Sangre Seca / Iduronidasa Límite: Female / Humans / Male Idioma: En Revista: Clin Biochem Año: 2017 Tipo del documento: Article Pais de publicación: Estados Unidos