Sharmistha Mitra, Ph.D. Titles and Appointments Assistant Professor Schools Medical School | Graduate School Departments Pediatrics | Biochemistry Graduate Programs Biological Chemistry, Genetics, Development and Disease Biography Sharmistha Mitra, Ph.D., is an Assistant Professor in the Department of Pediatrics, Division of Child Neurology and Department of Biochemistry at UT Southwestern Medical Center. She is also an investigator within the Peter O'Donnell Jr. Brain Institute. She joined UT Southwestern faculty as instructor in July 2020. Dr. Mitra earned a bachelor’s in Zoology at the University of Calcutta in Kolkata, India. She then completed a Bachelor of Science in Biological Sciences, Molecular, Cellular, and Developmental at the University of New Hampshire, graduating summa cum laude. In 2013, she completed a Doctor of Philosophy in Biological Sciences in the field of ubiquitin binding domains at Virginia Tech University. Dr. Mitra focuses much of her research on protein modification by ubiquitin and its impact on diseases. Especially, she is interested in identifying the role of ubiquitination in glycogen biology and its role in glycogen metabolism related neurological and neuromuscular diseases. Research Interest Protein-protein interactions in glycogen metabolism and neuromuscular diseases Role of ubiquitin ligases in glycogen storage diseases Therapy of glycogen metabolism-related pediatric neuromuscular diseases Publications Lafora disease gene therapy: EPM2A but not EPM2B overexpression results in Lafora body formation Alao EO, Sheibani M, Wu J, Marriam U, Evans D, Kasiri S, Verma M, Nitschke S, Nitschke F, Gray SJ, Mitra S, Minassian BA Neurotherapeutics 2026 Mar 23 Lithium exacerbates Lafora body formation in the Epm2a-/- Lafora disease mouse model Wu J, Lynn TC, Nitschke S, Mitra S, Minassian BA Neuroscience letters 2025 May 856-858 Myofiber-type-dependent ‘boulder’ or ‘multitudinous pebble’ formations across distinct amylopectinoses Mitra S, Chen B, Shelton JM, Nitschke S, Wu J, Covington L, Dear M, Lynn T, Verma M, Nitschke F, Fuseya Y, Iwai K, Evers BM, Minassian BA Acta Neuropathologica 2024 Jun 147 Laforin targets malin to glycogen in Lafora progressive myoclonus epilepsy Mitra S, Chen B, Wang P, Chown EE, Dear M, Guisso DR, Mariam U, Wu J, Gumusgoz E, Minassian BA DMM Disease Models and Mechanisms 2023 Jan 16 Glycogen synthase downregulation rescues the amylopectinosis of murine RBCK1 deficiency Nitschke S, Sullivan MA, Mitra S, Marchioni CR, Lee JP, Smith BH, Ahonen S, Wu J, Chown EE, Wang P, Petković S, Zhao X, Digiovanni LF, Perri AM, Israelian L, Grossman TR, Kordasiewicz H, Vilaplana F, Iwai K, Nitschke F, Minassian BA Brain 2022 Jul 145 2361-2377 Lafora disease: Current biology and therapeutic approaches Mitra S, Gumusgoz E, Minassian BA Revue Neurologique 2022 Apr 178 315-325 LUBAC: A new player in polyglucosan body disease Aboujaoude A, Minassian B, Mitra S Biochemical Society Transactions 2021 Nov 49 2443-2454 Targeting Gys1 with AAV‐SaCas9 Decreases Pathogenic Polyglucosan Bodies and Neuroinflammation in Adult Polyglucosan Body and Lafora Disease Mouse Models Gumusgoz E, Guisso DR, Kasiri S, Wu J, Dear M, Verhalen B, Nitschke S, Mitra S, Nitschke F, Minassian BA Neurotherapeutics 2021 Apr 18 1414-1425 Characterization of Staphylococcus epidermidis Polynucleotide phosphorylase and its interactions with ribonucleases RNase J1 and RNase J2 Raj R, Mitra S, Gopal B Biochemical and Biophysical Research Communications 2018 Jan 495 2078-2084 Six-Transmembrane Epithelial Antigen of Prostate 1 (STEAP1) Has a Single b Heme and Is Capable of Reducing Metal Ion Complexes and Oxygen Kim K, Mitra S, Wu G, Berka V, Song J, Yu Y, Poget S, Wang DN, Tsai AL, Zhou M Biochemistry 2016 Dec 55 6673-6684 Results 1-10 of 14 1 2 Next Last Honors & Awards Dr. Roy Elterman Pediatric Neurology Research Award (2020-2021) Nature Career Expo Travel AwardUniversity of Texas System (2018) Biophysical Society monthly newsletterSelected to be featured in the Biophysical Society Student Spotlight section (2012) Graduate Research & Development Grant AwardGraduate Student Association, Virginia Tech (2012) 'Make-a-difference' ScholarshipCollege of Science, Virginia Tech (2012-2013) The American Heart Association Predoctoral FellowshipThe American Heart Association (2012-2013) Best Interdisciplinary Oral Presentation AwardAmerican Coastal Conference Interdisciplinary Forum for Diversity in Life Sciences, Virginia Tech (2010) Certificate of achievement for 'Outstanding Participation in US Community and Campus life'US Department of State, Bureau of Educational and Cultural Affairs (2008) Certificate of Recognition for 'Outstanding Academic Achievement' Academy for Educational Development (2008) Fralin Graduate FellowshipFralin Research Institute, Virginia Tech (2008-2009) International Peace Scholarship for Women (2008-2009) Undergraduate Research AwardUniversity of New Hampshire, Undergraduate Research Opportunities Program (2007-2008) Partnership for Learning Undergraduate Studies Scholarship (PLUS)Scholarship created by the U.S Department of State's Bureau of Educational and Cultural Affairs, guided by Fulbright Association, India and funded by Academy for Educational Development, USA. Selected as the only Molecular biology student among ten scholars from India (2006) Special Medal Award Department of ZoologyBethune College, University of Calcutta for achieving First Class Honors in Zoology (2006) 2005 Gold Medal AwardBy Tadoba Andhari Tiger project (TATP), India for obtaining the 1st position in a national level debate on Molecular Biology organized by TATP (2005) Dean's listBethune College, Department of Zoology (2003-2006) 2001 Foundation for Excellence ScholarshipFor excellent performance in 10th standard (2001) Professional Associations/Affiliations American Society for Biochemistry and Molecular Biology (ASBMB) American Society of Cell Biology (ASCB) American Society of Investigative Pathology (ASIP)