Maria Guimaraes Da Lomba Ferraz
Analyst
- Name
- Dr. M.J. Guimaraes Da Lomba Ferraz
- Telephone
- 071 5276458
- m.j.ferraz@lic.leidenuniv.nl
Analyst
- Faculty of Science
- LIC
- Chemistry for Human Health
- Bio-organic Synthesis
- Gaspar P., Marques A.R.A., Guimaraes Da Lomba Ferraz M.J., Damme M., Kramer G., Mirzaian M., Gijbels M., Ottenhoff R., Roomen C. van, Overkleeft H.S., Schwake M., Heybrock S., Macario M.C., Saftig P. & Aerts J.M.F.G. (2025), LIMP-2 deficiency-associated glycolipid abnormalities in mice, Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids 1870(7): 159657.
- Marques A.R.A., Ferreira I.S., Ribeiro Q., Guimaraes Da Lomba Ferraz M.J., Lopes E., Pinto D., Hall M., Ramalho J., Artola Perez de Azanza M.E., Almeida M.S., Rodrigues G., Goncalves P.A., Ferreira J., Borbinha C., Marto J.P., Viana-Baptista M., Gouveiae e Melo R., Pedro L.M., Soares M.I.L., Vaz W.L.C., Vieira O.V. & Aerts J.M.F.G. (2025), Glucosylated cholesterol accumulates in atherosclerotic lesions and impacts macrophage immune response, Journal of Lipid Research 66(6): 100825.
- Katzy R.E., Neer R.H.P. van, Guimaraes Da Lomba Ferraz M.J., Nicolai K., Passioura T., Suga H., Jongkees S.A.K. & Artola Perez de Azanza M.E. (2025), Development of selective nanomolar cyclic peptide ligands as GBA1 enzyme stabilisers, RSC Chemical Biology 6(4): 563-570.
- Wheeler S., Bhardwaj M., Kenyon V., Guimaraes Da Lomba Ferraz M.J., Aerts J.M.F.G. & Sillence D.J. (2024), Mitochondrial dysfunction in NPC1-deficiency is not rescued by drugs targeting the glucosylceramidase GBA2 and the cholesterol-binding proteins TSPO and StARD1, FEBS Letters 598(4): 477-484.
- Koemans T.S., Bennett M., Guimaraes Da Lomba Ferraz M.J., Armstrong Z.W.B., Artola Perez de Azanza M.E., Aerts J.M.F.G., Codee J.D.C., Overkleeft H.S. & Davies G.J. (2024), Structure-guided design of C3-branched swainsonine as potent and selective human Golgi α-mannosidase (GMII) inhibitor, Chemical Communications 60(82): 11734-11737.
- Bannink S., Bila K.O., Weperen J. van, Ligthart N.A.M., Ferraz M.J., Boot R.G., Vliet D. van der, Boer D.E.C., Overkleeft H.S., Artola M. & Aerts J.M.F.G. (2024), 6-O-alkyl 4-methylumbelliferyl-β-D-glucosides as selective substrates for GBA1 in the discovery of glycosylated sterols, Journal of Lipid Research 65(11): 100670.
- Liefhebber J.M.P., Brasser G., Spronck E.A., Ottenhoff R., Paerels L., Guimaraes Da Lomba Ferraz M.J., Schwarz L.K., Efthymiopoulou N., Kuo C.-L., Montenegro-Miranda P.S., Evers M.M., Aerts J.M.F.G. & Liu Y.P. (2024), Preclinical efficacy and safety of adeno-associated virus 5 alpha-galactosidase: a gene therapy for Fabry disease, Molecular Therapy: Methods and Clinical Development 32(4): 101375.
- Gracht D. van der, Rowland R.J., Roig-Zamboni V., Guimaraes Da Lomba Ferraz M.J., Louwerse G.A.M., Geurink P.P., Aerts J.M.F.G., Sulzenbacher G., Davies G.J., Overkleeft H.S. & Artola Perez de Azanza M.E. (2023), Fluorescence polarisation activity-based protein profiling for the identification of deoxynojirimycin-type inhibitors selective for lysosomal retaining alpha- and beta-glucosidases, Chemical Science 14(34): 9136-9144.
- Moraitou M., Sotiroudis G., Papagiannakis N., Ferraz M.J., Xenakis A., Aerts J.M.F.G., Stefanis L. & Michelakakis H. (2023), a-Synuclein and lipids in erythrocytes of Gaucher disease carriers and patients before and after enzyme replacement therapy, PLoS ONE 18(2): e0277602.
- Papadopoulou P., Pol R. van der, Hilten N. van, Moradi M.A., Ferraz M.J., Aerts J.M.F.G., Sommerdijk N., Risselada H.J., Sevink G.J.A. & Kros A. (2023), Lipase-mediated selective hydrolysis of lipid droplets in phase separated-liposomes . ChemRXiv_Biological and Chemical Chemistry. [working paper].
- Su Q., Schroder S.P., Lelieveld L.T., Guimaraes Da Lomba Ferraz M.J., Verhoek M., Boot R.G., Overkleeft H.S., Aerts J.M.F.G., Artola Perez de Azanza M.E. & Kuo C.L. (2023), Corrigendum: Xylose-configured cyclophellitols as selective inhibitors for glucocerebrosidase, ChemBioChem 24(3): e202300728.
- Nami F., Joao Ferraz M., BakkumT., Aerts J.M.F.G. & Pandit A. (2022), Real‐Time NMR recording of fermentation and lipid metabolism processes in live microalgae cells, Angewandte Chemie (International Edition) : e202117521.
- Lelieveld L.T., Gerhardt S., Maas S., Zwiers K.C., Wit C. de, Beijk E.H., Guimaraes Da Lomba Ferraz M.J., Artola Perez de Azanza M.E., Meijer A.H., Tudorache C., Salvatori D., Boot R.G. & Aerts J.M.F.G. (2022), Consequences of excessive glucosylsphingosine in glucocerebrosidase-deficient zebrafish, Journal of Lipid Research 63(5): 100199.
- Katzy R.E., Guimaraes Da Lomba Ferraz M.J., Hazeu M.D., Overkleeft H.S. & Aerts J.M.F.G. (2022), In situ glucosylceramide synthesis and its pharmacological inhibition analysed in cells by 13C5-sphingosine precursor feeding and mass spectrometry, FEBS Letters 596(18): 2400-2408.
- Boer D.E., Mirzaian M., Ferraz M.J., Zwiers K.C., Baks M.V., Hazeu M.D., Ottenhoff R., Marques A.R.A., Meijer R., Roos J.C.P., Cox T.M., Boot R.G., Pannu N., Overkleeft H.S., Artola M. & Aerts J.M.F.G. (2021), Human glucocerebrosidase mediates formation of xylosyl-cholesterol by beta-xylosidase and transxylosidase reactions, Journal of Lipid Research 62: 100018.
- Su Q., Schröder S.P., Lelieveld L.T., Ferraz M.J., Verhoek M., Boot R.G., Overkleeft H.S., Aerts J.M.F.G., Artola M. & Kuo C.L. (2021), Xylose-configured cyclophellitols as selective inhibitors for glucocerebrosidase, ChemBioChem 22(21): 3090-3098.
- Dahl M., Smith E.M.K., Warsi S., Rothe M., Guimaraes De Lomba Ferraz M.J., Aerts J.M.F.G., Golipour A., Harper C., Pfeifer R., Pizzurro D., Schambach A., Mason C. & Karlsson S. (2021), Correction of pathology in mice displaying Gaucher disease type 1 by a clinically-applicable lentiviral vector, Molecular Therapy: Methods and Clinical Development 20: 312-323.
- Boer D.E.C., Mirzaian M., Ferraz M.J., Nadaban A., Schreuder A., Hovnanian A., Smeden J. van, Bouwstra J.A. & Aerts J.M.F.G. (2020), Glucosylated cholesterol in skin: synthetic role of extracellular glucocerebrosidase, Clinica Chimica Acta 510: 707-710.
- Boer D.E.C., Mirzaian M., Ferraz M.J., Zwiers K.C., Baks M.V., Hazeu M.D., Ottenhoff R., Marques A.R.A., Meijer R., Roos J.P.C., Cox T.M., Boot R.G., Pannu N., Overkleeft H.S., Artola M. & Aerts J.M.F.G. (2020), Human glucocerebrosidase mediates formation of xylosyl-cholesterol by β-xylosidase and transxylosidase reactions, Journal of Lipid Research 61(12): jlr.RA120001043.
- Eijk M. van, Ferraz M.J., Boot R.G. & Aerts J.M.F.G. (2020), Lyso-glycosphingolipids: presence and consequences, Essays in Biochemistry 64(3): 565-578.
- Lelieveld L.T., Mirzaian M., Kuo C.L., Artola Perez de Azanza M.E., Guimaraes da Lomba Ferraz M.J., Peter R.E.A., Akiyama H., Greimel P., Berg R.J.B.H.N. van den, Overkleeft H.S., Boot R.G., Meijer A. & Aerts J.M.F.G. (2019), Role of β-glucosidase 2 in aberrant glycosphingolipid metabolism: model of glucocerebrosidase deficiency in zebrafish, Journal of Lipid Research 60(11): 1851-1867.
- Artola M., Hedberg C., Rowland R.J., Raich L., Kytidou K., Wu L., Schaaf A., Ferraz M.J., Marel G.A. van der, Codee J.D.C., Rovira C., Aerts J.M.F.G., Davies G.J. & Overkleeft H.S. (2019), Alpha-d-Gal-cyclophellitol cyclosulfamidate is a Michaelis complex analog that stabilizes therapeutic lysosomal alpha-galactosidase A in Fabry disease, Chemical Science 10(40): 9233-9243.
- Wheeler S., Haberkant P., Bhardwaj M., Tongue P., Ferraz M.J., Halter D., Sprong H., Schmid R., Aerts J.M.F.G., Sullo N. & Sillence D.J. (2019), Cytosolic glucosylceramide regulates endolysosomal function in Niemann-Pick type C disease, Neurobiology of Disease 127: 242-252.
- Liefhebber J.M.P., Zon T. van der, Paerels L., Ferraz M.J., Ottenhoff R., Zancanella V., Au B., Kuo C.L., Vries C.J.M. de, Evers M.M., Konstantinova P., Aerts J.M.F.G., Deventer S.J. van & Liu Y.P. (2019), Development of an AAV5-Based Gene Therapy for Fabry Disease. CELL PRESS. [other].
- Cabasso O., Paul S., Dorot O., Maor G., Krivoruk O., Pasmanik-Chor M., Mirzaian M., Ferraz M.J., Aerts J.M.F.G. & Horowitz M. (2019), Drosophila melanogaster Mutated in its GBA1b Ortholog Recapitulates Neuronopathic Gaucher Disease, Journal of Clinical Medicine 8(9): 1420.
- Karlsson S., Smith E., Rothe M., Ferraz M.J., Aerts J.M.F.G., Golipour A., Harpe C., Pfeifer R., Pizzurro D., Schambach A. & Mason C. (2019), A Clinical Glucocerebrosidase Lentiviral Vector Corrects the Pathology and Clinical Signs in a Mouse Model for Type 1 Gaucher Disease. CELL PRESS. [other].
- Rakhshandehroo M., Eijkeren R.J. van, Gabriel T.L., Haar C. de, Gijzel S.M.W., Hamers N., Ferraz M.J., Aerts J.M.F.G., Schipper H.S., Eijk M.C. van, Boes M. & Kalkhoven E. (2019), Adipocytes harbor a glucosylceramide biosynthesis pathway involved in iNKT cell activation, Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids 1864(8): 1157-1167.
- Aerts J.M.F.G., Artola M.E., Eijk M.C. van, Ferraz M.J. & Boot R.G. (2019), Glycosphingolipids and infection. Potential new therapeutic avenues, Frontiers in Cell and Developmental Biology 7: 324.
- Schröder S.P., Wu L., Artola Perez de Azanza M.E., Hansen T., Offen W.A., Guimaraes da Lomba Ferraz M.J., Li K.-Y., Aerts J.M.F.G., Marel G.A. van der, Codee J.D.C., Davies G.J. & Overkleeft H.S. (2018), Gluco-1H-imidazole: A New Class of Azole-Type beta-Glucosidase Inhibitor, Journal of the American Chemical Society 140(15): 5045-5048.
- Kytidou K., Beekwilder J., Artola M., Meel E. van, Wilbers R.H.P., Moolenaar G.F., Goosen N., Ferraz M.J., Katzy R., Voskamp P., Florea B.I., Hokke C.H., Overkleeft H.S., Schots A., Bosch D., Pannu N. & Aerts J.M.F.G. (2018), Nicotiana benthamiana α-galactosidase A1.1 can functionally complement human α-galactosidase A deficiency associated with Fabry disease, Journal of Biological Chemistry 293(26): 10042-10058.
- Kaissarian N., Kang J., Shu L., Guimaraes da Lomba Ferraz M.J., Aerts J.M.F.G. & Shayman J.A. (2018), Dissociation of globotriaosylceramide and impaired endothelial function in α-galactosidase-A deficient EA.hy926 cells, Molecular Genetics and Metabolism 125(4): 338-344.
- Kytidou K., Beenakker T.J.M., Westerhof L.B., Hokke C.H., Moolenaar G.F., Goosen N., Mirzaian M., Guimaraes da Lomba Ferraz M.J., Geus M.A.R. de, Kallemeijn W.W., Overkleeft H.S., Boot R.G., Schots A., Bosch D. & Aerts J.M.F.G. (2017), Human Alpha Galactosidases Transiently Produced in Nicotiana benthamiana Leaves: New Insights in Substrate Specificities with Relevance for Fabry Disease, Frontiers in Plant Science 8: 1026.
- Mirzaian M., Wisse P., Guimaraes da Lomba Ferraz M.J., Marques A.R.A., Gaspar P., Oussoren S.V., Kytidou K., Codée J.D.C., Marel G.A. van der, Overkleeft H.S. & Aerts J.M.F.G. (2017), Simultaneous quantitation of sphingoid bases by UPLC-ESI-MS/MS with identical C-13-encoded internal standards, Clinica Chimica Acta 466: 178-184.
- Artola Perez de Azanza M.E., Wu L., Guimaraes da Lomba Ferraz M.J., Kuo C.L., Raich L., Breen I.Z., Offen W.A., Codee J.D.C., Marel G.A. van der, Rovira C., Aerts J.M.F.G., Davies G.J. & Overkleeft H.S. (2017), 1,6-Cyclophellitol Cyclosulfates: A New Class of Irreversible Glycosidase Inhibitor, ACS Central Science 3(7): 784-793.
- Beenakker T.J.M., Wander D.P.A., Offen W.A., Artola Perez de Azanza M.E., Raich L., Guimaraes da Lomba Ferraz M.J., Li K.Y., Houben J.H.P.M., Rijssel E.R. van, Hansen T., Marel G.A. van der, Codee J.D.C., Aerts J.M.F.G., Rovira C., Davies G.J. & Overkleeft H.S. (2017), Carba-cyclophellitols Are Neutral Retaining-Glucosidase Inhibitors, Journal of the American Chemical Society 139(19): 6534-6537.
- Aerts J.M.F.G., Guimaraes da Lomba Ferraz M.J., Mirzaian M., Gaspar P., Oussoren S.V., Wisse P., Kuo C.L., Lelieveld L.T., Kytidou K., Hazeu M.D., Boer D.E.C., Meijer R., Lienden M.J.C. van der, Herrera D., Gabriel T.L., Aten J., Overkleeft H.S., Eijk M.C. van, Boot R.G. & Rosa Alcalde Marques A. (2017), Lysosomal Storage Diseases. For Better or Worse: Adapting to Defective Lysosomal Glycosphingolipid Breakdown. In: , eLS. eLS: John Wiley & Sons, Ltd.. 1-13.
- Mirzaian M., Wisse P., Ferraz M.J., Marques A.R., Gabriel T.L., Roomen C.P. van, Ottenhoff R., Eijk M. van, Codée J.D., Marel G.A. van der, Overkleeft H.S. & Aerts J.M. (2016), Accurate quantification of sphingosine-1-phosphate in normal and Fabry disease plasma, cells and tissues by LC-MS/MS with (13)C-encoded natural S1P as internal standard, Clinica Chimica Acta 459: 36-44.
- Marques A.R., Mirzaian M., Akiyama H., Wisse P., Ferraz M.J., Gaspar P., Ghauharali-Van der Vlugt K., Meijer R., Giraldo P., Alfonso P., Irún P., Dahl M., Karlsson S., Pavlova E.V., Cox T.M., Scheij S., Verhoek M., Ottenhoff R., Roomen C.P. van, Pannu N.S., Van Eijk M., Dekker N., Boot R.G., Overkleeft H.S., Blommaart E., Hirabayashi Y. & Aerts J.M. (2016), Glucosylated cholesterol in mammalian cells and tissues: formation and degradation by multiple cellular β-glucosidases, 57: 451-463.
- Guimaraes da Lomba Ferraz M.J., Marques A.R., Appelman M.D., Verhoek M., Strijland A., Mirzaian M., Scheij S., Ouairy C.M.J., Lahav D., Wisse P., Overkleeft H.S., Boot R.G. & Aerts J.M.F.G. (2016), Lysosomal glycosphingolipid catabolism by acid ceramidase: formation of glycosphingoid bases during deficiency of glycosidases, FEBS Letters 590(6): 716-725.
- Guimaraes da Lomba Ferraz M.J., Marques A.R.A., Gaspar P., Mirzaian M., Roomen C. van, Ottenhoff R., Alfonso P., Irún P., Giraldo P., Wisse P., Sá Miranda C., Overkleeft H.S. & Aerts J.M. (2016), Lyso-glycosphingolipid abnormalities in different murine models of lysosomal storage disorders, Molecular Genetics and Metabolism 117(2): 186–193.
- Smid B.E., Ferraz M.J., Verhoek M., Mirzaian M., Wisse P., Overkleeft H.S., Hollak C.E. & Aerts J.M. (2016), Biochemical response to substrate reduction therapy versus enzyme replacement therapy in Gaucher disease type 1 patients, Orphanet Journal of Rare Diseases 11: 28.
- Ouairy C.M.J., Ferraz M.J., Boot R.G., Baggelaar M.P., Stelt M. van der, Appelman M., Marel G.A. van der, Florea B.I., Aerts J.M.F.G. & Overkleeft H.S. (2015), Development of an acid ceramidase activity-based probe, Chemical Communications 51(28): 6161-6163.
- Berg R.J.B.H.N. van der, Rijssel E.R. van, Ferraz M.J., Houben J., Strijland A., Donker-Koopman W.E., Wennekes T., Bonger K.M., Ghisaidoobe A.B.T., Hoogendoorn S., Marel G.A. van der, Codee J.D.C., Overkleeft H.S. & Aerts J.M.F.G. (2015), Synthesis and Evaluation of Hybrid Structures Composed of Two Glucosylceramide Synthase Inhibitors, ChemMedChem 10(12): 2042-2062.
- Wisse P., Gold H., Mirzaian M., Guimaraes Da Lomba Ferraz M.J., Lutteke G., Berg R.J.B.H.N. van den, Elst H. van den, Lugtenburg J., Marel G.A. van der, Aerts J.M.F.G., Codee J.D.C. & Overkleeft H.S. (2015), Synthesis of a panel of carbon-13-labelled (glyco)sphingolipids, European Journal of Organic Chemistry 2015(12): 2661-2677.
- Guimaraes da Lomba Ferraz M.J., Kallemeijn W.W., Mirzaian M., Herrera Moro D., Marques A., Wisse P., Boot R.G., Willems L.I., Overkleeft H.S. & Aerts J.M.F.G. (2014), Gaucher disease and Fabry disease: New markers and insights in pathophysiology for two distinct glycosphingolipidoses, Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids 1841(5): 811-825.
- Willems L.I., Beenakker T.J.M., Murray B., Scheij S., Kallemeijn W.W., Boot R.G., Verhoek M., Donker-Koopman W.E., Guimaraes da Lomba Ferraz M.J., Rijssel E.R. van, Florea B.I., Codee J.D.C., Marel G.A. van der, Aerts J.M.F.G. & Overkleeft H.S. (2014), Potent and Selective Activity-Based Probes for GH27 Human Retaining alpha-Galactosidases, Journal of the American Chemical Society 136(33): 11622-11625.
- Aerts J.M.F.G., Guimaraes da Lomba Ferraz M.J., Boot R.G., Breemen M.J. van, Dekker N., Kramer G., Hollak C.E.M., Maas M., Linthorst G.E., Smid B., Rombach S.M., Dussen L. van, Poorthuis B. & Groener J.E.M. (2013), Biomarkers for lysosomal storage disorders. In: Surendran S. (Ed.), Neurochemistry of metabolic diseases: lysosomal storage diseases, phenylketonuria, and Canavan disease. Hauppage, NY: Nova Science Publishers. 169-202.
- Aerts J.M.F.G., Kallemeijn W.W., Wegdam W., Joao Ferraz M., Van Breemen M.J., Dekker N., Kramer G., Poorthuis B.J., Groener J.E.M., Cox-Brinkman J., Rombach S.M., Hollak C.E.M., Linthorst G.E., Witte M.D., Gold H., Marel G.A. van der, Overkleeft H.S. & Boot R.G. (2011), Biomarkers in the diagnosis of lysosomal storage disorders: proteins, lipids, and inhibodies, Journal of Inherited Metabolic Disease 34(3): 605-619.