Use of a commercial agarose gel for analysis of urinary glycosaminoglycans in mucopolysaccharidoses
Braz. j. pharm. sci
; 52(4): 693-697, Oct.-Dec. 2016. tab, graf
Artigo
em Inglês
| LILACS
| ID: biblio-951881
Biblioteca responsável:
BR40.1
Localização: BR40.1
ABSTRACT
ABSTRACT Mucopolysaccharidoses (MPS) are a group of inherited metabolic disorders caused by deficiency of enzymes that degrade glycosaminoglycans (GAGs). Urinary excretion of GAGs is a common feature of MPS, and is considered their major biomarker. We aimed to adapt the GAG electrophoresis method to a commercial agarose gel which would be able to separate urinary GAGs in a simpler way with good sensitivity and reproducibility. Urine samples from patients previously diagnosed with MPS I, IV, and VI were used as electrophoretic standards. Samples from patients on enzyme replacement therapy (ERT) were also assessed. Commercial agarose gel electrophoresis was effective, showing proper definition and separation of GAG bands. Detection sensitivity exceeded 0.1 µg and band reproducibility were consistent. GAG bands quantified in urine samples from patients on ERT correlated very strongly (correlation coefficient = 0.98) with total GAG concentrations. This application of gel electrophoresis demonstrates the possibility of monitoring patients with MPS treated with ERT by analyzing separately the GAGs excreted in urine. We suggest this process should be applied to MPS screening as well as to follow-up of patients on treatment.
Texto completo:
Disponível
Coleções:
Bases de dados internacionais
Base de dados:
LILACS
Assunto principal:
Mucopolissacaridoses
/
Eletroforese em Gel de Ágar
/
Glicosaminoglicanos
Tipo de estudo:
Guia de prática clínica
Limite:
Criança, pré-escolar
/
Feminino
/
Humanos
/
Masculino
Idioma:
Inglês
Revista:
Braz. j. pharm. sci
Ano de publicação:
2016
Tipo de documento:
Artigo
País de afiliação:
Brasil
Instituição/País de afiliação:
Federal University of Rio Grande do Sul/BR