Positions
- Assistant Professor
-
Molecular and Human Genetics
Jan and Dan Duncan Neurological Research Institute
Baylor College of Medicine
Houston, TX US
Education
- BS from Univerisdad Autonoma
- 06/2001 - Madrid, Spain
- PhD from Universidad Autonoma
- 11/2007 - Madrid, Spain
- Postdoctoral Fellowship at Baylor College of Medicine
- Houston, United States
Websites
Selected Publications
- Al-Ramahi I, Lu B, Di Paola S, Pang K, de Haro M, Peluso I, Gallego-Flores T, Malik NT, Erikson K, Bleiberg BA, Avalos M, Fan G, Rivers LE, Laitman AM, Diaz-García JR, Hild M, Palacino J, Liu Z, Medina DL, Botas J. "High-Throughput Functional Analysis Distinguishes Pathogenic, Nonpathogenic, and Compensatory Transcriptional Changes in Neurodegeneration.." Cell Syst.. 2018 Jul; Pubmed PMID: 29936182
- Al-Ramahi I, Giridharan SSP, Chen YC, Patnaik S, Safren N, Hasegawa J, de Haro M, Wagner Gee AK, Titus SA, Jeong H, Clarke J, Krainc D, Zheng W, Irvine RF, Barmada S, Ferrer M, Southall N, Weisman LS, Botas J, Marugan JJ. "Inhibition of PIP4Kγ ameliorates the pathological effects of mutant huntingtin protein.." Elife. 2017 Dec; Pubmed PMID: 29256861
- Maxime W.C. Rousseaux, Gabriel E. Vázquez-Vélez, Ismael Al-Ramahi, Hyun-Hwan Jeong, Aleksandar Bajic, Jean-Pierre Revelli, Hui Ye, Emily T. Phan, Jennifer M. Deger, Alma M. Perez, Ji-Yoen Kim, Laura A. Lavery, Qikia Xu, Mamie Z. Li, et. al. "A druggable genome screen identifies modifiers of α-synuclein levels via a tiered cross-species validation approach." Journal of Neuroscience. 2018 Sep;
- Rousseaux MW, de Haro M, Lasagna-Reeves CA, De Maio A, Park J, Jafar-Nejad P, Al-Ramahi I, Sharma A, See L, Lu N, Vilanova-Velez L, Klisch TJ, Westbrook TF, Troncoso JC, Botas J, Zoghbi HY. "TRIM28 regulates the nuclear accumulation and toxicity of both alpha-synuclein and tau.." Elife. 2016 Oct; Pubmed PMID: 27779468
- Lasagna-Reeves CA, de Haro M, Hao S, Park J, Rousseaux MW, Al-Ramahi I, Jafar-Nejad P, Vilanova-Velez L, See L, De Maio A, Nitschke L, Wu Z, Troncoso JC, Westbrook TF, Tang J, Botas J, Zoghbi HY. "Reduction of Nuak1 Decreases Tau and Reverses Phenotypes in a Tauopathy Mouse Model.." Neuron. 2016 Oct; Pubmed PMID: 27720485
- Soriano S, Calap-Quintana P, Llorens JV, Al-Ramahi I, Gutiérrez L, Martínez-Sebastián MJ, Botas J, Moltó MD. "Metal Homeostasis Regulators Suppress FRDA Phenotypes in a Drosophila Model of the Disease.." PLoS One.. 2016 Jul; Pubmed PMID: 27433942
- Langfelder P, Cantle JP, Chatzopoulou D, Wang N, Gao F, Al-Ramahi I, Lu XH, Ramos EM, El-Zein K, Zhao Y, Deverasetty S, Tebbe A, Schaab C, Lavery DJ, Howland D, Kwak S, Botas J, Aaronson JS, Rosinski J, Coppola G, Horvath S, Yang XW. "Integrated genomics and proteomics define huntingtin CAG length-dependent networks in mice.." Nat Neurosci.. 2016 Apr; Pubmed PMID: 26900923
- El-Daher MT, Hangen E, Bruyère J, Poizat G, Al-Ramahi I, Pardo R, Bourg N, Souquere S, Mayet C, Pierron G, Lévêque-Fort S, Botas J, Humbert S, Saudou F. "Huntingtin proteolysis releases non-polyQ fragments that cause toxicity through dynamin 1 dysregulation.." Embo J. 2015 Jul; Pubmed PMID: 26165689
- Calap-Quintana P, Soriano S, Llorens JV, Al-Ramahi I, Botas J, Moltó MD, Martínez-Sebastián MJ. "TORC1 Inhibition by Rapamycin Promotes Antioxidant Defences in a Drosophila Model of Friedreich's Ataxia.." PLoS One.. 2015 Jul; Pubmed PMID: 26158631
- Yao Y, Cui X, Al-Ramahi I, Sun X, Li B, Hou J, Difiglia M, Palacino J, Wu ZY, Ma L, Botas J, Lu B. "A striatal-enriched intronic GPCR modulates huntingtin levels and toxicity.." Elife.. 2015 Mar; Pubmed PMID: 25738228
- Lu XH, Mattis VB, Wang N, Al-Ramahi I, van den Berg N, Fratantoni SA, Waldvogel H, Greiner E, Osmand A, Elzein K, Xiao J, Dijkstra S, de Pril R, Vinters HV, Faull R, Signer E, Kwak S, Marugan JJ, Botas J, Fischer DF, Svendsen CN, Munoz-Sanjuan I, Yang XW. "Targeting ATM ameliorates mutant Huntingtin toxicity in cell and animal models of Huntington's disease.." Sci Transl Med.. 2014 Dec; Pubmed PMID: 25540325
- Park J, Al-Ramahi I, Tan Q, Mollema N, Diaz-Garcia JR, Gallego-Flores T, Lu HC, Lagalwar S, Duvick L, Kang H, Lee Y, Jafar-Nejad P, Sayegh LS, Richman R, Liu X, Gao Y, Shaw CA, Arthur JS, Orr HT, Westbrook TF, Botas J, Zoghbi HY. "RAS-MAPK-MSK1 pathway modulates ataxin 1 protein levels and toxicity in SCA1.." Nature.. 2013 Jun; Pubmed PMID: 23719381
- de Haro M, Al-Ramahi I, Jones KR, Holth JK, Timchenko LT, Botas J. "Smaug/SAMD4A restores translational activity of CUGBP1 and suppresses CUG-induced myopathy.." PLoS Genet.. 2013 Apr; Pubmed PMID: 23637619
- Lu B, Al-Ramahi I, Valencia A, Wang Q, Berenshteyn F, Yang H, Gallego-Flores T, Ichcho S, Lacoste A, Hild M, Difiglia M, Botas J, Palacino J. "Identification of NUB1 as a suppressor of mutant Huntington toxicity via enhanced protein clearance.." Nat Neurosci.. 2013 May; Pubmed PMID: 23525043
- Miller JP, Yates BE, Al-Ramahi I, Berman AE, Sanhueza M, Kim E, de Haro M, DeGiacomo F, Torcassi C, Holcomb J, Gafni J, Mooney SD, Botas J, Ellerby LM, Hughes RE. "A genome-scale RNA-interference screen identifies RRAS signaling as a pathologic feature of Huntington's disease.." PLoS Genet.. 2012 Nov; Pubmed PMID: 23209424
- Shirasaki DI, Greiner ER, Al-Ramahi I, Gray M, Boontheung P, Geschwind DH, Botas J, Coppola G, Horvath S, Loo JA, Yang XW. "Network organization of the huntingtin proteomic interactome in mammalian brain.." Neuron.. 2012 Jul; Pubmed PMID: 22794259
- Zhang N, Li B, Al-Ramahi I, Cong X, Held JM, Kim E, Botas J, Gibson BW, Ellerby LM. "Inhibition of lipid signaling enzyme diacylglycerol kinase epsilon attenuates mutant huntingtin toxicity.." J Biol Chem.. 2012 Jun; Pubmed PMID: 22511757
- Miller JP, Holcomb J, Al-Ramahi I, de Haro M, Gafni J, Zhang N, Kim E, Sanhueza M, Torcassi C, Kwak S, Botas J, Hughes RE, Ellerby LM. "Matrix metalloproteinases are modifiers of huntingtin proteolysis and toxicity in Huntington's disease.." Neuron.. 2010 Jul; Pubmed PMID: 20670829
- de Haro M, Al-Ramahi I, Benito-Sipos J, López-Arias B, Dorado B, Veenstra JA, Herrero P. "Detailed analysis of leucokinin-expressing neurons and their candidate functions in the Drosophila nervous system.." Cell Tissue Res.. 2010 Feb; Pubmed PMID: 19941006
- Al-Ramahi I, Pérez AM, Lim J, Zhang M, Sorensen R, de Haro M, Branco J, Pulst SM, Zoghbi HY, Botas J. "dAtaxin-2 mediates expanded Ataxin-1-induced neurodegeneration in a Drosophila model of SCA1.." PLoS Genet.. 2007 Dec; Pubmed PMID: 18166084
- Branco J, Al-Ramahi I, Ukani L, Pérez AM, Fernandez-Funez P, Rincón-Limas D, Botas J. "Comparative analysis of genetic modifiers in Drosophila points to common and distinct mechanisms of pathogenesis among polyglutamine diseases.." Hum Mol Genet.. 2008 Feb; Pubmed PMID: 17984172
- Al-Ramahi I, Lam YC, Chen HK, de Gouyon B, Zhang M, Pérez AM, Branco J, de Haro M, Patterson C, Zoghbi HY, Botas J. "CHIP protects from the neurotoxicity of expanded and wild-type ataxin-1 and promotes their ubiquitination and degradation.." J Biol Chem.. 2006 Sep; Pubmed PMID: 16831871
- de Haro M, Al-Ramahi I, De Gouyon B, Ukani L, Rosa A, Faustino NA, Ashizawa T, Cooper TA, Botas J. "MBNL1 and CUGBP1 modify expanded CUG-induced toxicity in a Drosophila model of myotonic dystrophy type 1.." Hum Mol Genet.. 2006 Jul; Pubmed PMID: 16723374
Funding
- 2016-2019 Behavioral and cell-based complementary screens to identify druggable targets modulating tau protein levels - #5R21NS096395 Grant funding from NIH-NIA
- 2015 - Pioneer SCA Translational Research Award Grant funding from National Ataxia Foundation
- 2011 - Brain Disorders and Development Training Grant NIH Ruth L. Kirschstein National Research Service Award
- 2009 - John J. Wasmuth Postdoctoral Fellowship Hereditary Disease Foundation
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