Genetic and Small Molecule Modifiers of Lysosomal Function

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Project Website Internet-based social media

Project Website and Internet-based social media setup (Twitter, Facebook, LinkedIn).

Organization of a summer school in Chile

Pubblicazioni

Biophysical impact of sphingosine and other abnormal lipid accumulation in Niemann-Pick disease type C cell models

Autori: Carreira AC, Pokorna S, Ventura AE, Walker MW, Futerman AH, Lloyd-Evans E, Almeira RFM, Silva LC.
Pubblicato in: Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids, Numero Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids, Volume 1866, Numero 8, August 2021, 158944, 2021, ISSN 0006-3002
Editore: Biochimica et Biophysica Acta (BBA)
DOI: 10.1016/j.bbalip.2021.158944

c-Abl Inhibition Activates TFEB and Promotes Cellular Clearance in a Lysosomal Disorder

Autori: Contreras PS, Tapia PJ, González-Hódar L, Peluso I, Soldati C, Napolitano G, Matarese M, Heras M, Valls C, Martinez A, Balboa E, Castro J, Leal N, Platt FM, Sobota A, Winter D, Klein AD, Medina DL, Ballabio A, Alvarez A, Zanlungo S
Pubblicato in: iScience, Numero iScience VOLUME 23, ISSUE 11, 101691, NOVEMBER 20, 2020, 2020, ISSN 2589-0042
Editore: ISCIENCE
DOI: 10.1016/j.isci.2020.101691

Dengue virus targets RBM10 deregulating host cell splicing and innate immune response

Autori: Pozzi B, Bragado L, Mammi P, Torti MF, Gaioli N, Gebhard LG, Solá MEG, Vaz-Drago R, Iglesias NG, García CC, Gamarnik AV, Srebrow A.
Pubblicato in: Nucleic Acids Research, Numero Nucleic Acids Research, Volume 48, Numero 12, 09 July 2020, Pages 6824–6838, 2020, Pagina/e Pages 6824–6838, ISSN 1362-4962
Editore: Nucleic Acids Research
DOI: 10.1093/nar/gkaa340

Acetyl-leucine slows disease progression in lysosomal storage disorders.

Autori: Kaya E, Smith DA, Smith C, Morris L, Bremova-Ertl T, Cortina-Borja M, Fineran P, Morten KJ, Poulton J, Boland B, Spencer J, Strupp M, Platt FM..
Pubblicato in: Brain Communications, Numero Brain Communications, Volume 3, Numero 1, 2021, fcaa148,, 2020, ISSN 2632-1297
Editore: Brain Communications
DOI: 10.1093/braincomms/fcaa148

Identification of genetic modifiers of murine hepatic β-glucocerebrosidase activity.

Autori: Durán A, Rebolledo-Jaramillo B, Olguin V, Rojas-Herrera M, Heras M, Calderón JF, Zanlungo S, Priestman DA, Platt FM, Klein AD
Pubblicato in: Biochemistry and Biophysics Reports, Numero Biochemistry and Biophysics Reports, Volume 28, December 2021, 101105, 2021, ISSN 2405-5808
Editore: Biochemistry and Biophysics Reports
DOI: 10.1016/j.bbrep.2021.101105

c-Abl activates RIPK3 signaling in Gaucher disease

Autori: Yañez MJ, Campos F, Marín T, Klein AD, Futerman AH, Alvarez AR, Zanlungo S.
Pubblicato in: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, Numero Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease - Volume 1867, Numero 5, 1 May 2021, 166089, 2021, ISSN 0925-4439
Editore: Elsevier BV
DOI: 10.1016/j.bbadis.2021.166089

Beneficial Effects of Acetyl-DL-Leucine (ADLL) in a Mouse Model of Sandhoff Disease.

Autori: Kaya E, Smith DA, Smith C, Boland B, Strupp M, Platt FM
Pubblicato in: J Clin Med, Numero J. Clin. Med. 2020, 9(4), 1050, 2020, ISSN 2077-0383
Editore: Multidisciplinary Digital Publishing Institute (MDPI)
DOI: 10.3390/jcm9041050

Smaug1 membrane-less organelles respond to AMPK and mTOR and affect mitochondrial function

Autori: Fernández-Alvarez AJ, Gabriela Thomas M, Pascual ML, Habif M, Pimentel J, Corbat AA, Pessoa JP, La Spina PE, Boscaglia L, Plessis A, Carmo-Fonseca M, Grecco HE, Casado M, Boccaccio GL.
Pubblicato in: J Cell Sci, Numero J Cell Sci. (2022);135(1):jcs253591, 2022, ISSN 0021-9533
Editore: The Company of Biologists Ltd.
DOI: 10.1242/jcs.253591

Cholesteryl Hemiazelate Induces Lysosome Dysfunction and Exocytosis in Macrophages

Autori: Domingues N, Calado RDA, Brito PH, Matthiesen R, Ramalho J, Soares MIL, Pereira T, Oliveira L, Vicente JR, Wong LH, Cho SM, Simões ICM, Sampaio J, Klose C, Surma MA, Almeida MS, Rodrigues G, Araújo-Gonçalves P, Ferreira J, Simons K, Pinho e Melo TMVD, Peden A, Almeida CG, Futter CE, Futerman AH, Vaz WLC, Vieira OV
Pubblicato in: bioRxiv, Numero bioRxiv, 2021, ISSN 2692-8205
Editore: bioRxiv
DOI: 10.1101/2021.01.05.422575

SUMO conjugation regulates the activity of the Integrator complex

Autori: Bragado L, Magalnik M, Mammi P, Romero A, Gaioli N, Pozzi B, Srebrow A.
Pubblicato in: bioRxiv, Numero bioRxiv 2022.03.07.483123 (under final review in Nucleic Acids Research), 2022, ISSN 2692-8205
Editore: bioRxiv
DOI: 10.1101/2022.03.07.483123

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