Paul Sampognaro, MD
Assistant Professor
Neurology
School of Medicine

Grants and Projects

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• TDP43 Degradation by the Lysosomal Proteases in Amyotrophic Lateral Sclerosis, NIH, 2022-2027

Publications (18)

Top publication keywords:
Gallic AcidElectronicsLysosomesOrganometallic CompoundsFrontotemporal DementiaDNA-Binding ProteinsMyoclonusCell PhoneBrain DiseasesC9orf72 ProteinPeptide HydrolasesBismuthNeurodegenerative DiseasesGastrointestinal Diseasesalpha-Synuclein

• Lysosomal protease-mediated APP degradation is pH-dependent, mutation-sensitive, and facilitates tau proteolysis.
  Molecular neurodegeneration advances 2026 Ackley C, Liau Z, Arya S, Antee T, Cheang E, Knudsen GM, Lane-Donovan C, Sampognaro PJ, Kao AW
• Prosaposin Is Cleaved Into Saposins by Multiple Cathepsins in a Progranulin-Regulated Fashion.
  Journal of neurochemistry 2026 Hodul M, Lane-Donovan C, Cheang ES, Gao V, Sampognaro PJ, Mambou EA, Yang Z, Kao AW
• Lysosomal Protease-Mediated APP Degradation is pH-Dependent, Mutation-Sensitive, and Facilitates Tau Proteolysis.
  Research square 2025 Ackley C, Liau Z, Arya S, Antee T, Knudsen GM, Lane-Donovan C, Sampognaro PJ, Kao AW
• Apilimod dimesylate in C9orf72 amyotrophic lateral sclerosis: a randomized phase 2a clinical trial.
  Brain : a journal of neurology 2024 Babu S, Nicholson KA, Rothstein JD, Swenson A, Sampognaro PJ, Pant P, Macklin EA, Spruill S, Paganoni S, Gendron TF, Prudencio M, Petrucelli L, Nix D, Landrette S, Nkrumah E, Fandrick K, Edwards J, …
• Correction: Mutations in α-synuclein, TDP-43 and tau prolong protein half-life through diminished degradation by lysosomal proteases.
  Molecular neurodegeneration 2023 Sampognaro PJ, Arya S, Knudsen GM, Gunderson EL, Sandoval-Perez A, Hodul M, Bowles K, Craik CS, Jacobson MP, Kao AW

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• Mutations in α-synuclein, TDP-43 and tau prolong protein half-life through diminished degradation by lysosomal proteases.
  Molecular neurodegeneration 2023 Sampognaro PJ, Arya S, Knudsen GM, Gunderson EL, Sandoval-Perez A, Hodul M, Bowles K, Craik CS, Jacobson MP, Kao AW
• Processing of progranulin into granulins involves multiple lysosomal proteases and is affected in frontotemporal lobar degeneration.
  Molecular neurodegeneration 2021 Mohan S, Sampognaro PJ, Argouarch AR, Maynard JC, Welch M, Patwardhan A, Courtney EC, Zhang J, Mason A, Li KH, Huang EJ, Seeley WW, Miller BL, Burlingame A, Jacobson MP, Kao AW
• Progranulin Adsorbs to Polypropylene Tubes and Disrupts Functional Assays: Implications for Research, Biomarker Studies, and Therapeutics.
  Frontiers in neuroscience 2020 Gururaj S, Sampognaro PJ, Argouarch AR, Kao AW
• Neuroglial stem cell-derived inflammatory pseudotumor (n-SCIPT): clinicopathologic characterization of a novel lesion of the lumbosacral spinal cord and nerve roots following intrathecal allogeneic stem cell intervention.
  Acta neuropathologica 2019 Sloan EA, Sampognaro PJ, Junn JC, Chin C, Jacques L, Ramachandran PS, DeRisi JL, Wilson MR, Kriegstein AR, Bollen AW, Solomon DA, Margeta M, Engstrom JW
• C9orf72-specific phenomena associated with frontotemporal dementia and gastrointestinal symptoms in the absence of TDP-43 aggregation.
  Acta neuropathologica 2019 Sampognaro PJ, Vatsavayai SC, Cosme CG, Hwang JL, Nolan A, Huang EJ, Seeley WW, De May MG
• Acute Sensorimotor Deficit as a Lone Manifestation of Neurocysticercosis: An Important Differential Diagnosis in Emergency Departments of Developed Countries.
  Journal of global infectious diseases 2019 Deng H, Yue JK, Sampognaro PJ, Rosendale N
• Bismuth Subgallate Toxicity in the Age of Online Supplement Use.
  The neurologist 2017 Sampognaro P, Vo KT, Richie M, Blanc PD, Keenan K
• EphA7 signaling guides cortical dendritic development and spine maturation.
  Proceedings of the National Academy of Sciences of the United States of America 2014 Clifford MA, Athar W, Leonard CE, Russo A, Sampognaro PJ, Van der Goes MS, Burton DA, Zhao X, Lalchandani RR, Sahin M, Vicini S, Donoghue MJ
• Medical student appraisal: electronic resources for inpatient pre-rounding.
  Applied clinical informatics 2013 Sampognaro PJ, Mitchell SL, Weeks SR, Khalifian S, Markman TM, Uebel LW, Dattilo JR
• Medical student appraisal: applications for bedside patient education.
  Applied clinical informatics 2013 Markman TM, Sampognaro PJ, Mitchell SL, Weeks SR, Khalifian S, Dattilo JR
• Survival and recurrence for patients undergoing surgery of skull base intracranial metastases.
  Journal of neurological surgery. Part B, Skull base 2013 Chaichana KL, Flores M, Acharya S, Sampognaro P, Bettegowda C, Rigamonti D, Weingart JD, Olivi A, Gallia GL, Brem H, Lim M, Quinones-Hinojosa A
• Medical student appraisal: searching on smartphones.
  Applied clinical informatics 2013 Khalifian S, Markman T, Sampognaro P, Mitchell S, Weeks S, Dattilo J
• Histone deacetylase inhibition suppresses myogenin-dependent atrogene activation in spinal muscular atrophy mice.
  Human molecular genetics 2012 Bricceno KV, Sampognaro PJ, Van Meerbeke JP, Sumner CJ, Fischbeck KH, Burnett BG