Zobrazeno 1 - 8
of 8
pro vyhledávání: '"Ângela M. Sgaravatti"'
Autor:
Caroline Paula Mescka, Ângela M. Sgaravatti, Carlos Severo Dutra-Filho, Fernanda Rech Zanin, Clovis Milton Duval Wannmacher, Alessandra Selinger Magnusson, Andrea Pereira Rosa, Amanda Szekir de Oliveira, Angela T. S. Wyse, Moacir Wajner, Carolina Didonet Pederzolli
Publikováno v:
Metabolic Brain Disease. 24:415-425
Tyrosine levels are abnormally elevated in tissues and physiological fluids of patients with inborn errors of tyrosine catabolism especially in tyrosinemia type II which is caused by deficiency of tyrosine aminotransferase (TAT) and provokes eyes, sk
Autor:
Nicoli T. Henn, Fernanda Rech Zanin, Ângela M. Sgaravatti, Eline Coan Romagna, Francieli J Rockenbach, Carlos Severo Dutra-Filho, Moacir Wajner, Clovis Milton Duval Wannmacher, Angela T. S. Wyse, Ângela de Mattos Dutra, Carolina Didonet Pederzolli
Publikováno v:
Metabolic Brain Disease. 24:283-298
N-acetylaspartic acid (NAA) is the biochemical hallmark of Canavan Disease, an inherited metabolic disease caused by deficiency of aspartoacylase activity. NAA is an immediate precursor for the enzyme-mediated biosynthesis of N-acetylaspartylglutamic
Autor:
Carlos Severo Dutra-Filho, Moacir Wajner, Clovis Milton Duval Wannmacher, Mirian Bonaldi Sgarbi, Alessandra Selinger Magnusson, Katia Bueno Deckmann, Caroline Paula Mescka, Carolina Didonet Pederzolli, Angela T. S. Wyse, Evelise de Souza Streck, Ângela M. Sgaravatti
Publikováno v:
Metabolic brain disease. 25(2)
N-Acetylaspartic acid accumulates in Canavan Disease, a severe inherited neurometabolic disease clinically characterized by severe mental retardation, hypotonia, macrocephaly and generalized tonic and clonic type seizures. Considering that the mechan
Autor:
Angela T. S. Wyse, Mirian Bonaldi Sgarbi, Carlos Severo Dutra-Filho, Carmen Regla Vargas, Carolina Didonet Pederzolli, Bernardo Remuzzi Zandoná, Caroline Paula Mescka, Clovis Milton Duval Wannmacher, Daniella de Moura Coelho, Ângela M. Sgaravatti, Moacir Wajner
Publikováno v:
Metabolic brain disease. 25(2)
5-Oxoproline accumulates in glutathione synthetase deficiency, an autossomic recessive inherited disorder clinically characterized by hemolytic anemia, metabolic acidosis, and severe neurological symptoms whose mechanisms are poorly known. In the pre
Autor:
Caroline Paula Mescka, Clovis Milton Duval Wannmacher, Alessandra Selinger Magnusson, Fernanda Rech Zanin, Carlos Severo Dutra-Filho, Mirian Bonaldi Sgarbi, Moacir Wajner, Amanda Szekir de Oliveira, Ângela M. Sgaravatti, Angela T. S. Wyse, Carolina Didonet Pederzolli
Publikováno v:
Metabolic brain disease. 24(2)
gamma-Hydroxybutyric acid (GHB) is a naturally occurring compound in the central nervous system (CNS) whose tissue concentration are highly increased in the neurometabolic-inherited deficiency of succinic semialdehyde dehydrogenase (SSADH) activity o
Autor:
Katia Bueno Deckmann, Ângela M. Sgaravatti, Bernardo Remuzzi Zandoná, Carolina Didonet Pederzolli, Francieli J Rockenbach, Tarsila Barros Moraes, Moacir Wajner, José Maria Monserrat, Carlos Severo Dutra-Filho, Clovis Milton Duval Wannmacher, Bethânia Andrade de Vargas, Mirian Bonaldi Sgarbi, Angela T. S. Wyse
Publikováno v:
International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience. 26(6)
Tyrosine accumulates in inborn errors of tyrosine catabolism, especially in tyrosinemia type II, where tyrosine levels are highly elevated in tissues and physiological fluids of affected patients. In tyrosinemia type II, high levels of tyrosine are c
Autor:
Gustavo da Costa Ferreira, Marcos Luiz Santos Perry, Vanessa Grando, Rafael Borba Rosa, Carlos Severo Dutra-Filho, Moacir Wajner, César A. J. Ribeiro, Patrícia Fernanda Schuck, Ângela M. Sgaravatti, Anna Laura Schmidt
Publikováno v:
Neurochemical research. 33(1)
In the present work we investigated the in vitro effect of the branched-chain amino acids (BCAA) accumulating in maple syrup urine disease (MSUD) on some parameters of energy metabolism in cerebral cortex of rats. 14CO2 production from [1-14C]acetate
Autor:
Ivan Cunha Bustamante-Filho, Adriana Pires Neves, Fernanda Rech Zanin, Carlos Severo Dutra-Filho, Carolina Didonet Pederzolli, R.C. Mattos, Ângela M. Sgaravatti, M.I.M. Jobim
Publikováno v:
Animal Reproduction Science. 107:312
I.C. Bustamante-Filho a,b,∗, C.D. Pederzolli b, F.R. Zanin b, Â.M. Sgaravatti b, A.P. Neves a,c, C.S. Dutra-Filho b, M.I. Mascarenhas Jobim a, R.C. Mattos a a REPROLAB, Faculdade de Veterinaria, Universidade Federal do Rio Grande do Sul, Porto Ale