Udai Pandey, Ph.D.

  • Associate Professor
  • Pediatrics (Primary) and Human Genetics (Secondary)

Education & Training

  • B.S.-Dr. RML Avadh University, India, Biochemistry and Microbiology
  • M.S.-Dr. RML Avadh University, India, Biochemistry
  • Ph.D.-Sanjay Gandhi Postgraduate Institute of Medical Sciences, India:Medical Genetics

Research Interest Summary

Molecular Mechanisms of Human Neurodegenerative Diseases

Research Categories

Research Interests

The focus of Pandey laboratory is to elucidate the molecular mechanisms of amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, and other related motor neurons diseases. Recently, several RNA-binding proteins have been found to be mutated in both sporadic and familial forms of ALS. Disease causing mutations in these RNA binding proteins suggest that underlying defects in RNA metabolism might play an important role in causing motor neuron degeneration in ALS. The Pandey lab developed fly models of ALS that recapitulate several key pathological features of human disease such as neurodegeneration and behavioral defects. We are utilizing cell biological, genetic and biochemical techniques to understand molecular mechanisms of ALS in drosophila and mammalian neuronal models. The Pandey lab is looking for genetic and small molecule modifiers of ALS in fly and mammalian neuronal models. We expect that these modifiers will not only allow us to understand molecular basis of ALS but also help in developing therapeutic interventions for ALS.

Representative Publications

Fortuna TR, Kour S, Anderson EN, Ward C, Rajasundaram D, Donnelly CJ, Wyne H, Shewmaker F, Pandey UB. DDX17 is involved in DNA damage repair and modifies FUS toxicity in an RGG-domain dependent manner. Acta Neuropathol. 2021 June 1. doi: 10.1007/s00401-021-02333-z 

Anderson EN, Morera AA, Kour S, Cherry JD, Ramesh N, Gleixner A, Schwartz JC, Ebmeier C, Old W, Donnelly CJ, Cheng JP, Kline AE, Kofler J, Stein TD, Pandey UB. Traumatic injury compromises nucleocytoplasmic transport and leads to TDP-43 pathology. eLife. 2021 May 26;10:e67587. PMID: 34060470

Kour S, Rajan DS, Fortuna TR, Anderson EN, Ward C, Lee Y, Lee S, Shin YB, Chae JH, Choi M, Siquier K, Cantagrel V, Amiel J, Stolerman ES, Barnett SS, Cousin MA, Castro D, McDonald K, Kirmse B, Nemeth AH, Rajasundaram D, Innes AM, Lynch D, Frosk P, Collins A, Gibbons M, Yang M, Desguerre I, Boddaert N, Gitiaux C, Rydning SL, Selmer KK, Urreizti R, Garcia-Oguiza A, Osorio AN, Verdura E, Pujol A, McCurry HR, Landers JE, Agnihotri S, Andriescu EC, Moody SB, Phornphutkul C, Sacoto MJG, Begtrup A, Houlden H, Kirschner J, Schorling D, Rudnik-Schöneborn S, Strom TM, Leiz S, Juliette K, Richardson R, Yang Y, Zhang Y, Wang M, Wang J, Wang X, Platzer K, Donkervoort S, Bönnemann CG, Wagner M, Issa MY, Elbendary HM, Stanley V, Maroofian R, Gleeson JG, Zaki MS, Senderek J, Pandey UB. Loss of function mutations in GEMIN5 cause a neurodevelopmental disorder. Nature Comm. 2021 May 7;12(1):2558. 

Lin YC, Kumar MS, Ramesh N, Anderson EN, Nguyen AT, Kim B, Cheung S, McDonough JA, Skarnes WC, Lopez-Gonzalez R, Landers JE, Fawzi NL, Mackenzie IRA, Lee EB, Nickerson JA, Grunwald D, Pandey UB, Bosco DA. Interactions between ALS-linked FUS and nucleoporins are associated with defects in the nucleocytoplasmic transport pathway. Nature Neurosci. 2021 May 31. doi: 10.1038/s41593-021-00859-9. Epub ahead of print. PMID: 34059832

Anderson A, Gochenaur L, Singh A, Grant R, Patel K, Watkins S, Wu J, Pandey UB. Traumatic injury induces Stress Granule Formation and enhances Motor Dysfunctions in ALS Models. Hum Mol Genet. 2018 Apr 15;27(8):1366-1381

Bakthavachalu B, Huelsmeier J, Sudhakaran I, Hillebrand J, Singh A, Petrauskas A, Thiagarajan D, Sankaranarayanan M, Mizoe L, Anderson EA, Pandey UB, Ross E, VijayRaghavan K, Parker R, Ramaswami M. RNP-granules assembly via Ataxin02 disordered domains is required for long­ term memory and neurodegeneration. Neuron. 2018 May 16;98(4):754-766 *Previewed in 'Neuron'

Guo L, Kim HJ, Wang H, Monaghan J, Freyermuth F, Sung JC, O'Donovan K, Fare CM, Diaz Z, Singh N, Zhang ZC, Coughlin M, Sweeny EA, DeSantis ME, Jackrel ME, Rodell CB, Burdick JA, King OD, Gitler AD, Lagier-Tourenne C, Pandey UB, Chook YM, Taylor JP, Shorter J. Nuclear-Import Receptors Reverse Aberrant Phase Transitions of RNA-Binding Proteins with Prion-like Domains. Cell. 2018 Apr 19;173(3):677-692

Marrone L, Japtok J, Reinhardt P, Janosch A, Andree A, Lee H, Moebius C, Reinhardt L, Hackmann K, Klink B, Alberti S, Bickle M, Hyman AA, Casci I, Pandey UB, Hermann A, and Sterneckert J. Phenotypic screening using iPSC reporter lines identifies brain-penetrant drugs stimulating autophagy as therapeutics for FUS-ALS. Stem Cell Reports 2018 Feb 13;10(2):375- 389

Daigle JG, Krishnamurthy K, Ramesh R, Casci I, Monaghan J, McAvoy K, Godfrey GW, Daniel D, Johnson E, Monahan Z, Shewmaker F, Pasinelli P, Pandey UB. Pur alpha ameliorates FUS toxicity and regulates cytoplasmic stress granule dynamics. Acta Neuropathol. 2016 Apr;l31{4):605-20

Scaramuzzino C, Casci I, Parodi S, Lievens PMJ, Milioto C, Polanco MJ, Chivet M, Mishra A, Badders N, Aggarwal N, Grunseich C, Sambataro F, Basso M, Fackelmayer FO, Taylor JP, Pandey UB and Pennuto M. Protein arginine methyltransferase 6 enhances polyglutamine-expanded androgen receptor function and toxicity in spinal and bulbar muscular atrophy. Neuron 2015 Jan 7; 84(7)

Wen X, Tan W, Westergard T, Krishnamurthy K, Shamamandri Markandaiah S, Lin S, lchida JK, Monaghan J, Pandey UB, Pasinelli P and Trotti D. Antisense Praline-Arginine RAN dipeptides linked to C9ORF72-ALS/FTD form toxic nuclear aggregates that initiate in vitro and in vivo neuronal death. Neuron 2014 Dec 17;84(6)

Daigle JG, Lanson NA, Smith RB, Casci I, Maltare A, Monaghan J, Nichols CD, Kryndushkin D, Shewmaker F, Pandey UB. RNA binding ability of FUS regulates neurodegeneration, cytoplasmic mislocalization and incorporation into stress granules associated with FUS carrying ALS-linked mutations. Human Molecular Genetics 2013 Mar 15;22(6):1193-205.

Lanson NA, Maltare A, King H, Smith R, Kim JH, Taylor JP, Lloyd TE and Pandey UB. A Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43. Human Molecular Genetics 2011 Jul 1;20(13):2510-23.

Pandey UB, Nie Z, Batlevi Y, McCray BA, Ritson GP, Nedelsky NB, Schwartz SL, DiProspero NA, Knight MA, Schuldiner 0, Padmanabhan R, Hild M, Berry DL, Garza D, Hubbert CC, Yao TP, Baehrecke EH, Taylor JP. HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS. Nature. 2007 Jun 14; 447(7146):860-4. *Previewed in Neuron and selected for 'must read paper' by faculty 1000 biology.

Full List of Publications