Research Overview

• RNA modification and translation regulation
• Neurodevelopmental disorders linked to RNA function
• Biochemistry and molecular biology

Our laboratory investigates the cellular roles of nucleic acid modification enzymes in biological processes ranging from neurodevelopment to the cellular stress response. In particular, we focus on discovering the targets and functions of two classes of enzymes: the SAM-dependent methyltransferases and the iron-dependent AlkB dioxygenases. To study the diverse processes modulated by these enzymes, we use an integrated biochemical, molecular and genetic approach in mammalian tissue culture systems as well as mouse knockout models. Through this approach, we have discovered novel targets and functions for enzymes involved in DNA repair, RNA modification and regulated cell death. The pathways and mechanisms identified through our studies provide critical insight into multiple aspects of human health and disease, including anti-cancer chemotherapy, degenerative disorders and aging.

Research Interests

• RNA modification and translation regulation
• Neurodevelopmental disorders linked to RNA function
• Biochemistry and molecular biology

Recent Publications

Jenna M. Lentini, Hessa S. Alsaif, Eissa Faqeih, Fowzan S. Alkuraya, Dragony Fu DALRD3 encodes a protein mutated in epileptic encephalopathy that targets arginine tRNAs for 3-methylcytosine modification Nature Communications, 2020 https://doi.org/10.1038/s41467-020-16321-6

Felix Hagelskamp, Kayla Borland, Jillian Ramos, Alan G Hendrick, Dragony Fu, et al. Broadly applicable oligonucleotide mass spectrometry for the analysis of RNA writers and erasers in vitro Nucleic Acids Research, 2020 https://doi.org/10.1093/nar/gkaa091

Kejia Zhang, Jenna M. Lentini, Christopher T. Prevost, Mais O. Hashem, Fowzan S. Alkuraya, et al. An intellectual disability‐associated missense variant in TRMT1 impairs tRNA modification and reconstitution of enzymatic activity Human Mutation, 2020 https://doi.org/10.1002/humu.23976

Biallelic variants in CTU2 cause DREAM-PL syndrome and impair thiolation of tRNA wobble U34. Human mutation, 2019 https://doi.org/10.1002/humu.23870

Jillian Ramos, Lu Han, Yan Li, Felix Hagelskamp, Stefanie M. Kellner, et al. Formation of tRNA Wobble Inosine in Humans Is Disrupted by a Millennia-Old Mutation Causing Intellectual Disability Molecular and Cellular Biology, 2019 https://doi.org/10.1128/MCB.00203-19

Jillian Ramos, Dragony Fu The emerging impact of tRNA modifications in the brain and nervous system Biochimica et Biophysica Acta (BBA) - Gene Regulatory Mechanisms, 2019 https://doi.org/10.1016/j.bbagrm.2018.11.007

Chen Gu, Jillian Ramos, Ulrike Begley, Peter C. Dedon, Dragony Fu, et al. Phosphorylation of human TRM9L integrates multiple stress-signaling pathways for tumor growth suppression Science Advances, 2018 https://doi.org/10.1126/sciadv.aas9184

Jenna M. Lentini, Jillian Ramos, Dragony Fu Monitoring the 5-methoxycarbonylmethyl-2-thiouridine (mcm5s2U) modification in eukaryotic tRNAs via the γ-toxin endonuclease RNA, 2018 https://doi.org/10.1261/rna.065581.118

Padgett LR, Lentini JM, Holmes MJ, Stilger KL, Fu D, et al. Elp3 and RlmN: A tale of two mitochondrial tail-anchored radical SAM enzymes in Toxoplasma gondii. PloS one, 2018 https://doi.org/10.1371/journal.pone.0189688

Joshua M. Dewe, Benjamin L. Fuller, Jenna M. Lentini, Stefanie M. Kellner, Dragony Fu TRMT1-Catalyzed tRNA Modifications Are Required for Redox Homeostasis To Ensure Proper Cellular Proliferation and Oxidative Stress Survival Molecular and Cellular Biology, 2017 https://doi.org/10.1128/MCB.00214-17

For more, please visit my ORCID Profile