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1.

Allosteric inhibition of the IRE1α RNase preserves cell viability and function during endoplasmic reticulum stress.

Ghosh, Rajarshi; Wang, Likun; Wang, Eric S; Perera, B Gayani K; Igbaria, Aeid; Morita, Shuhei; Prado, Kris; Thamsen, Maike; Caswell, Deborah; Macias, Hector; Weiberth, Kurt F; Gliedt, Micah J; Alavi, Marcel V; Hari, Sanjay B; Mitra, Arinjay K; Bhhatarai, Barun; Schürer, Stephan C; Snapp, Erik L; Gould, Douglas B; German, Michael S; Backes, Bradley J; Maly, Dustin J; Oakes, Scott A; Papa, Feroz R.

Cell ; 158(3): 534-48, 2014 Jul 31.

Artículo en Inglés | MEDLINE | ID: mdl-25018104

2.

Recent advances in, and challenges of, designing OMA1 drug screens.

Alavi, Marcel V.

Pharmacol Res ; 176: 106063, 2022 02.

Artículo en Inglés | MEDLINE | ID: mdl-34999225

3.

Targeted OMA1 therapies for cancer.

Alavi, Marcel V.

Int J Cancer ; 145(9): 2330-2341, 2019 11 01.

Artículo en Inglés | MEDLINE | ID: mdl-30714136

4.

Long-term photoreceptor rescue in two rodent models of retinitis pigmentosa by adeno-associated virus delivery of Stanniocalcin-1.

Roddy, Gavin W; Yasumura, Douglas; Matthes, Michael T; Alavi, Marcel V; Boye, Sanford L; Rosa, Robert H; Fautsch, Michael P; Hauswirth, William W; LaVail, Matthew M.

Exp Eye Res ; 165: 175-181, 2017 12.

Artículo en Inglés | MEDLINE | ID: mdl-28974356

5.

Aging and Vision.

Alavi, Marcel V.

Adv Exp Med Biol ; 854: 393-9, 2016.

Artículo en Inglés | MEDLINE | ID: mdl-26427437

6.

Type IV Collagens and Basement Membrane Diseases: Cell Biology and Pathogenic Mechanisms.

Mao, Mao; Alavi, Marcel V; Labelle-Dumais, Cassandre; Gould, Douglas B.

Curr Top Membr ; 76: 61-116, 2015.

Artículo en Inglés | MEDLINE | ID: mdl-26610912

7.

A clinically complex form of dominant optic atrophy (OPA8) maps on chromosome 16.

Carelli, Valerio; Schimpf, Simone; Fuhrmann, Nico; Valentino, Maria Lucia; Zanna, Claudia; Iommarini, Luisa; Papke, Monika; Schaich, Simone; Tippmann, Sabine; Baumann, Britta; Barboni, Piero; Longanesi, Lora; Rugolo, Michela; Ghelli, Anna; Alavi, Marcel V; Youle, Richard J; Bucchi, Laura; Carroccia, Rosanna; Giannoccaro, Maria Pia; Tonon, Caterina; Lodi, Raffaele; Cenacchi, Giovanna; Montagna, Pasquale; Liguori, Rocco; Wissinger, Bernd.

Hum Mol Genet ; 20(10): 1893-905, 2011 May 15.

Artículo en Inglés | MEDLINE | ID: mdl-21349918

8.

OMA1 High-Throughput Screen Reveals Protease Activation by Kinase Inhibitors.

Alavi, Marcel V.

ACS Chem Biol ; 16(11): 2202-2211, 2021 11 19.

Artículo en Inglés | MEDLINE | ID: mdl-34672515

9.

Tau phosphorylation and OPA1 proteolysis are unrelated events: Implications for Alzheimer's Disease.

Alavi, Marcel V.

Biochim Biophys Acta Mol Cell Res ; 1868(12): 119116, 2021 11.

Artículo en Inglés | MEDLINE | ID: mdl-34400172

10.

OMA1-An integral membrane protease?

Alavi, Marcel V.

Biochim Biophys Acta Proteins Proteom ; 1869(2): 140558, 2021 02.

Artículo en Inglés | MEDLINE | ID: mdl-33130089

11.

A splice site mutation in the murine Opa1 gene features pathology of autosomal dominant optic atrophy.

Alavi, Marcel V; Bette, Stefanie; Schimpf, Simone; Schuettauf, Frank; Schraermeyer, Ulrich; Wehrl, Hans F; Ruttiger, Lukas; Beck, Susanne C; Tonagel, Felix; Pichler, Bernd J; Knipper, Marlies; Peters, Thomas; Laufs, Juergen; Wissinger, Bernd.

Brain ; 130(Pt 4): 1029-42, 2007 Apr.

Artículo en Inglés | MEDLINE | ID: mdl-17314202

12.

Individuals with progranulin haploinsufficiency exhibit features of neuronal ceroid lipofuscinosis.

Ward, Michael E; Chen, Robert; Huang, Hsin-Yi; Ludwig, Connor; Telpoukhovskaia, Maria; Taubes, Ali; Boudin, Helene; Minami, Sakura S; Reichert, Meredith; Albrecht, Philipp; Gelfand, Jeffrey M; Cruz-Herranz, Andres; Cordano, Christian; Alavi, Marcel V; Leslie, Shannon; Seeley, William W; Miller, Bruce L; Bigio, Eileen; Mesulam, Marek-Marsel; Bogyo, Matthew S; Mackenzie, Ian R; Staropoli, John F; Cotman, Susan L; Huang, Eric J; Gan, Li; Green, Ari J.

Sci Transl Med ; 9(385)2017 04 12.

Artículo en Inglés | MEDLINE | ID: mdl-28404863

13.

Col4a1 mutations cause progressive retinal neovascular defects and retinopathy.

Alavi, Marcel V; Mao, Mao; Pawlikowski, Bradley T; Kvezereli, Manana; Duncan, Jacque L; Libby, Richard T; John, Simon W M; Gould, Douglas B.

Sci Rep ; 6: 18602, 2016 Jan 27.

Artículo en Inglés | MEDLINE | ID: mdl-26813606

14.

In Vivo Visualization of Endoplasmic Reticulum Stress in the Retina Using the ERAI Reporter Mouse.

Alavi, Marcel V; Chiang, Wei-Chieh; Kroeger, Heike; Yasumura, Douglas; Matthes, Michael T; Iwawaki, Takao; LaVail, Matthew M; Gould, Douglas B; Lin, Jonathan H.

Invest Ophthalmol Vis Sci ; 56(11): 6961-70, 2015 Oct.

Artículo en Inglés | MEDLINE | ID: mdl-26513501

15.

Strain-Dependent Anterior Segment Dysgenesis and Progression to Glaucoma in Col4a1 Mutant Mice.

Mao, Mao; Smith, Richard S; Alavi, Marcel V; Marchant, Jeffrey K; Cosma, Mihai; Libby, Richard T; John, Simon W M; Gould, Douglas B.

Invest Ophthalmol Vis Sci ; 56(11): 6823-31, 2015 Oct.

Artículo en Inglés | MEDLINE | ID: mdl-26567795

16.

Dominant optic atrophy, OPA1, and mitochondrial quality control: understanding mitochondrial network dynamics.

Alavi, Marcel V; Fuhrmann, Nico.

Mol Neurodegener ; 8: 32, 2013 Sep 25.

Artículo en Inglés | MEDLINE | ID: mdl-24067127

17.

Down-regulation of OPA1 alters mouse mitochondrial morphology, PTP function, and cardiac adaptation to pressure overload.

Piquereau, Jerome; Caffin, Fanny; Novotova, Marta; Prola, Alexandre; Garnier, Anne; Mateo, Philippe; Fortin, Dominique; Huynh, Le Ha; Nicolas, Valérie; Alavi, Marcel V; Brenner, Catherine; Ventura-Clapier, Renée; Veksler, Vladimir; Joubert, Frédéric.

Cardiovasc Res ; 94(3): 408-17, 2012 Jun 01.

Artículo en Inglés | MEDLINE | ID: mdl-22406748

18.

Electrophysiological and histologic assessment of retinal ganglion cell fate in a mouse model for OPA1-associated autosomal dominant optic atrophy.

Heiduschka, Peter; Schnichels, Sven; Fuhrmann, Nico; Hofmeister, Sabine; Schraermeyer, Ulrich; Wissinger, Bernd; Alavi, Marcel V.

Invest Ophthalmol Vis Sci ; 51(3): 1424-31, 2010 Mar.

Artículo en Inglés | MEDLINE | ID: mdl-19834041

19.

Solving a 50 year mystery of a missing OPA1 mutation: more insights from the first family diagnosed with autosomal dominant optic atrophy.

Fuhrmann, Nico; Schimpf, Simone; Kamenisch, York; Leo-Kottler, Beate; Alexander, Christiane; Auburger, Georg; Zrenner, Eberhart; Wissinger, Bernd; Alavi, Marcel V.

Mol Neurodegener ; 5: 25, 2010 Jun 14.

Artículo en Inglés | MEDLINE | ID: mdl-20546606

20.

Subtle neurological and metabolic abnormalities in an Opa1 mouse model of autosomal dominant optic atrophy.

Alavi, Marcel V; Fuhrmann, Nico; Nguyen, Huu Phuc; Yu-Wai-Man, Patrick; Heiduschka, Peter; Chinnery, Patrick F; Wissinger, Bernd.

Exp Neurol ; 220(2): 404-9, 2009 Dec.

Artículo en Inglés | MEDLINE | ID: mdl-19815013

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Allosteric inhibition of the IRE1α RNase preserves cell viability and function during endoplasmic reticulum stress.

Recent advances in, and challenges of, designing OMA1 drug screens.

Targeted OMA1 therapies for cancer.

Long-term photoreceptor rescue in two rodent models of retinitis pigmentosa by adeno-associated virus delivery of Stanniocalcin-1.

Aging and Vision.

Type IV Collagens and Basement Membrane Diseases: Cell Biology and Pathogenic Mechanisms.

A clinically complex form of dominant optic atrophy (OPA8) maps on chromosome 16.

OMA1 High-Throughput Screen Reveals Protease Activation by Kinase Inhibitors.

Tau phosphorylation and OPA1 proteolysis are unrelated events: Implications for Alzheimer's Disease.

OMA1-An integral membrane protease?

A splice site mutation in the murine Opa1 gene features pathology of autosomal dominant optic atrophy.

Individuals with progranulin haploinsufficiency exhibit features of neuronal ceroid lipofuscinosis.

Col4a1 mutations cause progressive retinal neovascular defects and retinopathy.

In Vivo Visualization of Endoplasmic Reticulum Stress in the Retina Using the ERAI Reporter Mouse.

Strain-Dependent Anterior Segment Dysgenesis and Progression to Glaucoma in Col4a1 Mutant Mice.

Dominant optic atrophy, OPA1, and mitochondrial quality control: understanding mitochondrial network dynamics.

Down-regulation of OPA1 alters mouse mitochondrial morphology, PTP function, and cardiac adaptation to pressure overload.

Electrophysiological and histologic assessment of retinal ganglion cell fate in a mouse model for OPA1-associated autosomal dominant optic atrophy.

Solving a 50 year mystery of a missing OPA1 mutation: more insights from the first family diagnosed with autosomal dominant optic atrophy.

Subtle neurological and metabolic abnormalities in an Opa1 mouse model of autosomal dominant optic atrophy.