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	Provedor de dados BJMBR (1) Autor Tutor,J.C. (1) Palavra-chave Activation energy (1) Biochemical characterization (1) GM2 gangliosidosis B1 variant (1) SS-Hexosaminidase isoenzymes (1) Tipo do documento Info:eu-repo/semantics/article (1) Ano 2004 (1) País Brazil (1) Idioma Inglês (1)		Ordenar por:  Relevância Autor Título Ano Registros recuperados: 1 Primeira ... 1 ... Última Biochemical characterization of the GM2 gangliosidosis B1 variant BJMBR Tutor,J.C.. The deficiency of the A isoenzyme of ß-hexosaminidase (Hex) produced by different mutations of the gene that codes for the alpha subunit (Tay-Sachs disease) has two variants with enzymological differences: the B variant consists of the absence of Hex A isoenzyme and the B1 variant produces an inactive Hex A isoenzyme for the hydrolysis of the GM2 ganglioside and synthetic substrates with negative charge. In contrast to the early childhood form of the B variant, the B1 variant appears at a later clinical stage (3 to 7 years of age) with neurodegenerative symptoms leading to the death of the patient in the second decade of life. The most frequent mutation responsible for the GM2 gangliosidosis B1 variant is R178H, which has a widespread geographic and ethnic... Tipo: Info:eu-repo/semantics/article Palavras-chave: GM2 gangliosidosis B1 variant; SS-Hexosaminidase isoenzymes; Activation energy; Biochemical characterization. Ano: 2004 URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2004000600001 Registros recuperados: 1 Primeira ... 1 ... Última

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