Publications

Below are selected works from the lab. See a full list on Dr. Wong’s Google Scholar page.

TDP-43 Cryptic Exons as Biomarkers

  • Irwin KE, Sheth U, Wong PC*, Gendron TF*. Fluid biomarkers for amyotrophic lateral sclerosis: a review. Mol Neurodegener 19, 9 (2024). DOI: 10.1186/s13024-023-00685-6 *co-corresponding author
  • Irwin KE, Jasin P, Braunstein KE, Sinha IR, Garret MA, Bowden KD, Chang K, Troncoso JC, Moghekar A, Oh ES, Raitcheva D, Bartlett D, Miller T, Berry JD, Traynor BJ, Ling JP, Wong PC. A fluid biomarker reveals loss of TDP-43 splicing repression in presymptomatic ALS–FTD. Nat Med 30, 382–393 (2024). DOI: 10.1038/s41591-023-02788-5

Biology and Pathophysiology of TDP-43

  • Baghel MS, Burns GD, Tsapatsis M, Mallika AP, Cruz ALF, Cao T, Chen XK, Rosa I, Marx SR, Ye Y, Sun S, Li T, Wong PC. Depletion of TDP-43 exacerbates tauopathy-dependent brain atrophy by sensitizing vulnerable neurons to caspase 3-mediated endoproteolysis of tau in a mouse model of Multiple Etiology Dementia. bioRxiv (2024). DOI: 10.1101/2024.06.26.600814 [Preprint]
  • Sinha IR, Sandal PS, Burns GD, Mallika AP, Irwin KE, Cruz ALF, Wang V, Rodríguez JL, Wong PC, Ling JP. Large-scale RNA-seq mining reveals ciclopirox triggers TDP-43 cryptic exons. bioRxiv (2024). DOI: 10.1101/2024.03.27.587011 [Preprint]
  • Donde A, Sun M, Ling JP, Braunstein KE, Pang B, Wen X, Cheng X, Chen L, Wong PC. Splicing repression is a major function of TDP-43 in motor neurons. Acta Neuropathol 138, 813-826 (2019). DOI: 10.1007/s00401-019-02042-8
  • Jeong YH, Ling JP, Lin SZ, Donde AN, Braunstein KE, Majounie E, Traynor BJ, LaClair KD, Lloyd TE, Wong PC. Tdp-43 cryptic exons are highly variable between cell types. Mol Neurodegener 1, 13 (2017). DOI: 10.1186/s13024-016-0144-x
  • Ling JP, Chhabra R, Merran JD, Schaughency PM, Wheelan SJ, Corden JL, Wong PC. PTBP1 and PTBP2 Repress Nonconserved Cryptic Exons. Cell Rep 17, 104-113 (2016). DOI: 10.1016/j.celrep.2016.08.071
  • Ling JP, Pletnikova O, Troncoso JC, Wong PC. TDP-43 repression of nonconserved cryptic exons is compromised in ALS-FTD. Science 349, 650-655 (2015). DOI: 10.1126/science.aab0983
  • Chiang PM, Ling JP, Jeong YH, Price DL, Aja SM, Wong PC. Deletion of TDP-43 down-regulates Tbc1d1, a gene linked to obesity, and alters body fat metabolism. PNAS 107, 16320-16324 (2010). DOI: 10.1073/pnas.1002176107

Validation of TDP-43 splicing repression as a therapeutic target

  • Donde A, Sun M, Jeong YH, Wen X, Ling JP, Lin SZ, Braunstein K, Nie S, Wang S, Chen L, Wong PC. Upregulation of ATG7 attenuates motor neuron dysfunction associated with depletion of TARDBP/TDP-43. Autophagy 16, 672-682 (2020). DOI: 10.1080/15548627.2019.1635379
  • Donde A, Sun M, Ling JP, Braunstein KE, Pang B, Wen X, Cheng X, Chen L, Wong PC. Splicing repression is a major function of TDP-43 in motor neurons. Acta Neuropathol 138, 813-826 (2019). DOI: 10.1007/s00401-019-02042-8
  • Sun M, Bell W, LaClair KD, Ling JP, Han H, Kageyama Y, Pletnikova O, Troncoso JC, *Wong PC, *Chen LL. Cryptic exon incorporation occurs in Alzheimer’s brain lacking TDP-43 inclusion but exhibiting nuclear clearance of TDP-43. Acta Neuropathologica 133, 923-931 (2017). DOI: 10.1007/s00401-017-1701-2 *equal contribution

Loss of TDP-43 splicing repression in skeletal muscle of Inclusion Body Myositis

  • Britson KA, Ling JP, Braunstein KE, Montagne J, Kastenschmidt J, Tsao W, Russel KA, Reed N, Wagner KR, Ostrow LW, Corse AM, Mammen AL, Villalta A, Larman HB, Wong PC*, Lloyd TE*. Loss of TDP-43 function and rimmed vacuoles persist after T cell depletion in a xenograft model of sporadic inclusion body myositis. Science Transl Med 14, eabi9196 (2022). DOI: 10.1126/scitranslmed.abi9196 *co-corresponding author