Research Interest

  • Age-related macular degeneration
  • Chemical biology
  • Drug discovery
  • Extracellular matrix proteins
  • Inherited retinal dystrophies
  • Ocular protein misfolding diseases
  • Stress-responsive signaling

Publications

Featured Publications LegendFeatured Publications

A novel H395R mutation in MKKS/BBS6 causes retinitis pigmentosa and polydactyly without other findings of Bardet-Biedl or McKusick-Kaufman syndrome.
Hulleman JD, Nguyen A, Ramprasad VL, Murugan S, Gupta R, Mahindrakar A, Angara R, Sankurathri C, Mootha VV Mol. Vis. 2016 22 73-81
Differential tolerance of 'pseudo-pathogenic' tryptophan residues in calcium-binding EGF domains of short fibulin proteins.
Nguyen A, Hulleman JD Exp. Eye Res. 2015 Jan 130 66-72
ATF6 activation reduces the secretion and extracellular aggregation of destabilized variants of an amyloidogenic protein.
Chen JJ, Genereux JC, Qu S, Hulleman JD, Shoulders MD, Wiseman RL Chem. Biol. 2014 Nov 21 11 1564-74
Genetic ablation of N-linked glycosylation reveals two key folding pathways for R345W fibulin-3, a secreted protein associated with retinal degeneration.
Hulleman JD, Kelly JW FASEB J. 2014 Nov
Unfolded protein response activation reduces secretion and extracellular aggregation of amyloidogenic immunoglobulin light chain.
Cooley CB, Ryno LM, Plate L, Morgan GJ, Hulleman JD, Kelly JW, Wiseman RL Proc. Natl. Acad. Sci. U.S.A. 2014 Aug
The systemic amyloid precursor transthyretin (TTR) behaves as a neuronal stress protein regulated by HSF1 in SH-SY5Y human neuroblastoma cells and APP23 Alzheimer's disease model mice.
Wang X, Cattaneo F, Ryno L, Hulleman J, Reixach N, Buxbaum JN J. Neurosci. 2014 May 34 21 7253-65
A high-throughput cell-based Gaussia luciferase reporter assay for identifying modulators of fibulin-3 secretion.
Hulleman JD, Brown SJ, Rosen H, Kelly JW J Biomol Screen 2013 Jul 18 6 647-58
Translational attenuation differentially alters the fate of disease-associated fibulin proteins.
Hulleman JD, Balch WE, Kelly JW FASEB J. 2012 Nov 26 11 4548-60
Effect of single amino acid substitution on oxidative modifications of the Parkinson's disease-related protein, DJ-1.
Madian AG, Hindupur J, Hulleman JD, Diaz-Maldonado N, Mishra VR, Guigard E, Kay CM, Rochet JC, Regnier FE Mol. Cell Proteomics 2012 Feb 11 2 M111.010892
Compromised mutant EFEMP1 secretion associated with macular dystrophy remedied by proteostasis network alteration.
Hulleman JD, Kaushal S, Balch WE, Kelly JW Mol. Biol. Cell 2011 Dec 22 24 4765-75

Books

Featured Books Legend Featured Books

Alpha-Synuclein self-assembly in Parkinson's disease: Insights from biophysical analyses. In Protein Misfolding in Biology and Disease

Rochet JC and Hulleman JD (2009). Trivandrum, India, Research Signpost

Honors & Awards

  • Bright Focus Foundation
    Macular Degeneration Research Award (2018-2020)
  • National Institutes of Health
    R01EY027785 (2018-2023)
  • National Institutes of Health
    R21EY028261 (2017-2019)
  • Bright Focus Foundation
    Macular Degeneration Research Award (2016-2018)
  • Karl Kirchgessner Foundation
    Vision Research Grant (2016-2019)
  • Research to Prevent Blindness
    Career Development Award (2015-2019)