Ana Paula Costa dos Santos Peralta Leandro
PhD (2001) in Pharmacy (Biochemistry), Universidade de Lisboa
Assistant Professor, Department of Biochemistry and Human Biology
Faculdade de Farmácia, Universidade de Lisboa — Av. Prof. Gama Pinto, 1649-003 Lisboa, Portugal
Paula research aims to develop new therapeutic approaches for Inborn Errors of Metabolism (IEM) either by replacement of the deficient enzyme or by rescuing the misfolded target enzyme using small compounds. With this aim a comprehensive set of experimental approaches to produce and biochemically/biophysically characterize native and variant proteins were established. Recently, Leandro interests expanded to the study of proteostasis networks aiming identify new candidate molecules to restore protein homeostasis in IEM.
Leandro J, Saraste J, Leandro P, Flatmark T. PKU mutation p.G46S prevents the stereospecific binding of L-Phe to the dimer of human phenylalanine hydroxylase regulatory domain. FEBS Open Bio 2017; 7:195-203.
Bonito C, Nunes J, Leandro J, Louro F, Leandro P, Ventura FV, Guedes RC. Unveiling the pathogenic molecular mechanisms of the most common variant (p.K329E) in Medium-chain acyl-CoA dehydrogenase deficiency by in vitro and in silico approaches. Biochem 2016; 55:7086-7098.
Mendes MIS, Smith DEC, Vicente JB, Tavares de Almeida I, Ben-Omran T, Salomons GS, Rivera IA, Blom HJ*, Leandro P*. Small aminothiol compounds improve the function of Arg to Cys variant proteins: effect on the human cystathionine β-synthase p.R336C. Hum Mol Genet 2015; 24: 7339-48 (*joint last authors).
Mendes MI, Santos AS, Smith DE, Lino PR, Colaço HG, de Almeida IT, Vicente JB, Salomons GS, Rivera I, Blom HJ, Leandro P. Insights into the regulatory domain of cystathionine Beta-synthase: characterization of six variant proteins. Hum Mutat 2014; 35:1195-202.
Montalbano F, Leandro J Farias G, Lino PR, Guedes RC, Vicente JB, Leandro P*, Gois PMP*. Phenylalanine iminoboronates as new phenylalanine hydroxylase modulators. RSC Advances 2014; 4: 61022-61027 (*joint corresponding authors).