Mineralogy and chemistry of the green stone artifacts (muiraquitãs) of the museums of the Brazilian State of Pará

Os muiraquitãs foram considerados de proveniência asiática ou, também, como peças esculpidas pelas lendárias mulheres guerreiras, as Amazonas. São peças, hoje, muito raras, encontradas em alguns acervos de Museus. Estudos mineralógicos e químicos de 23 peças do acervo dos Museus de Gemas e do Encontro em Belém, Brasil, mostraram que os muiraquitãs podem ser constituídos, tanto de quartzo, como de albita, ou microclínio, pirofilita, variscita, anortita e tremolita (equivalente ao jade nefrítico), minerais frequentes em formações geológicas do Brasil. No entanto quatro peças são constituídas de jadeíta, ou seja, em jade jadeítico, raro e desconhecido na Amazônia e Brasil. A constatação da presença desse mineral reacende a discussão em torno da origem mineralógica dos muiraquitãs encontrados na Amazônia. Essa origem, antes da atual descoberta, era defendida como amazônica, devido à ausência de jade jadeítico nas peças pesquisadas e pelo fato de jadeíta não ter sido encontrada no Brasil, mas na América Central e na Ásia.

Mucopolysaccharidoses in northern Brazil: targeted mutation screening and urinary glycosaminoglycan excretion in patients undergoing enzyme replacement therapy

Mucopolysaccharidoses (MPS) are rare lysosomal disorders caused by the deficiency of specific lysosomal enzymes responsible for glycosaminoglycan (GAG) degradation. Enzyme Replacement Therapy (ERT) has been shown to reduce accumulation and urinary excretion of GAG, and to improve some of the patients' clinical signs. We studied biochemical and molecular characteristics of nine MPS patients (two MPS I, four MPS II and three MPS VI) undergoing ERT in northern Brazil. The responsiveness of ERT was evaluated through urinary GAG excretion measurements. Patients were screened for eight common MPS mutations, using PCR, restriction enzyme tests and direct sequencing. Two MPS I patients had the previously reported mutation p.P533R. In the MPS II patients, mutation analysis identified the mutation p.R468W, and in the MPS VI patients, polymorphisms p.V358M and p.V376M were also found. After 48 weeks of ERT, biochemical analysis showed a significantly decreased total urinary GAG excretion in patients with MPS I (p < 0.01) and MPS VI (p < 0.01). Our findings demonstrate the effect of ERT on urinary GAG excretion and suggest the adoption of a screening strategy for genotyping MPS patients living far from the main reference centers.