In Preparation/Under Review
Gomez, A.E., Alvarado, K., Yase, R. A., Hsu, Y-H., Hulleman, J.D., and Kwon, R.Y. (2024) Dominant nonsense mutations in efemp1 alter vertebral and craniofacial characteristics in adult zebrafish. Published as a preprint |
2024
Mendoza, M.M., Poole, K., DiCesare, S.M., Nakahara, E., Bhatt, M.P., Hulleman, J.D., and Petroll, W.M. (2024) The role of vimentin in human corneal fibroblast spreading and myofibroblast transformation. |
Daniel, S., and Hulleman, J.D. Ω . Exploring ocular fibulin-3 (EFEMP1): anatomical, age-related, and species perspectives. [In Press, BBA: Molecular Basis of Disease.] ( Ω - corresponding author) PMID: 38750770 |
DiCesare, S.M., Ortega, A.J., Collier, G.E., Daniel, S., Thompson, K.N., McCoy, M.K., Posner, B.A., and Hulleman, J.D. Ω GSK3 inhibition reduces ECM protein production and prevents pathology associated with an early onset form of age-related macular degeneration. [In Press, Journal of Clinical Investigation - Insight. Published as a preprint] ( Ω - corresponding author) |
Daniel, S., Ortega, A.J., and Hulleman, J.D. Ω RPE basal lamina biology and pathophysiology relevant to age- related macular degeneration. Advances in Experimental Medicine and Biology. [In Press] ( Ω - corresponding author) |
Lankford, K.P., and Hulleman, J.D. Ω Protocol for HiBiT tagging endogenous proteins using CRISPR/Cas9 gene editing. STAR Protocols. 5(2):103000 ( Ω - corresponding author). PMID: 38598333 |
Zegeye, Y., Aredo, B., Yuksel, S., He, Y-G., Tang, M., Li, X., DiCesare, S.M., Hulleman, J.D., Gautron, L., Ludwig, S., Moresco, E.M., Beutler, B.A., Ufret-Vincenty, R.L. (2024) Deficiency of Herc3, an E3 ubiquitin ligase, leads to accumulation of subretinal microglia and retinal degeneration. Scientific Reports. 14(1):3010 PMID: 38321224 |
2023
Hayes, M.H., Woodard, D.R., and Hulleman, J.D. Ω. Ocular amyloid, condensates, and aggregates – higher-order protein assemblies participate in both retinal degeneration and function. Advances in Experimental Medicine and Biology. 1415: 263-267 PMID: 37440043 |
Yuksel, S., Aredo, B., Zegeye, Y., Zhao, C., Tang, M., Li, X., Hulleman, J.D., Gautron, L., Ludwig, S., Moresco, E.M., Butovich, I.A., Beutler, B.A., and Ufret-Vincenty, R.L. (2023). Forward genetic screening using fundus spot scale identifies an essential role for Lipe in retinal homeostasis. Communications Biology. 6(533) PMID: 37198396 |
2022
Burdon, K.P., Graham, P., Hadler, J., Hulleman, J.D., Prasutto, F., Boese, E.A., Craig, J.E., Fingert, J., Hewitt, A.W., J.D. Hulleman 4 Siggs, O.M., Whisenhunt, K., Young, T.L., Mackey, D.A., Dubowsky, A., and Souzeau, E. (2022). Specifications of the ACMG/AMP variant curation guidelines for MYOC: recommendations from the ClinGen Glaucoma Expert Panel. Human Mutation. 43(12):2170-2186. Selected as an Editor’s Choice article. PMID: 36217948 |
Nakahara, E., Mullapudi, V., Collier, G.E., Joachimiak, L.A., and Hulleman, J.D. Ω. A new DHFR-based destabilizing domain with enhanced turnover and broader flexibility. ACS Chemical Biology. 17(10):2877-2889 ( Ω - corresponding author). PMID: 36122928 |
Woodard, D.R., Daniel, S., Nakahara, E., Abbas, A., DiCesare, S.M., Collier, G.E., and Hulleman, J.D. Ω. A loss-of- function cysteine mutant in fibulin-3 (EFEMP1) forms aberrant extracellular disulfide-linked homodimers and alters extracellular matrix composition. Human Mutation. 43(12):1945-1955 ( Ω - corresponding author). Selected as an Editor’s Choice article. PMID: 35998264 |
Peng, H.*, Ramadurgum, P.*, Woodard, D.R.*, Daniel, S., Renwick, M., Nakahara, E., Datta, S., Chen, B., Aredo, B., Ufret-Vincenty, R.L. and Hulleman, J.D. Ω. Utility of the DHFR-based destabilizing domain across mouse models of retinal degeneration. iScience. 25(5):104206 (*- equal contribution, Ω - corresponding author). PMID: 35521529 |
Nakahara, E., and Hulleman, J.D. Ω. A simple secretion assay for assessing new and existing myocilin variants. Current Eye Research. Mar. 25; 1-5 ( Ω - corresponding author). PMID: 35196929 |
2021
Manna, M.S., Tamer, Y.T., Gaszek, I., Poulides, N., Ahmed, A., Wang, X., Toprak, F.C.R., Woodard, D.R., Koh, A.Y., Williams, N., Borek, D., Atilgan, A.R., Hulleman, J.D., Atilgan, C., Tambar, U.K., and Toprak, E. A trimethoprim derivative impedes antibiotic resistance evolution. Nature Communications. 12(1): 2949. Featured Article in Nat. Comm. PMID: 34011959 |
Woodard, D.R., Xing, C., Ganne, P., Liang, H., Mahindrakar, A., Sankurathri, C., Hulleman, J.D. # , and Mootha, V.V. # A novel homozygous P388S mutation in TULP1 causes protein instability and retinitis pigmentosa. Molecular Vision 27: 179-190 ( # - co-corresponding author) PMID: 33907372 |
Woodard, D.R., Nakahara, E., and Hulleman, J.D. Ω. Clinically-identified C-terminal mutations in fibulin-3 are prone to misfolding and destabilization. Scientific Reports. 11(1): 2998. ( Ω -corresponding author) PMID: 33542268 |
2020
Daniel, S., Renwick, M., Datta, S., Chau, V.Q., Zode, G.S., Wade, E.M., Robertson, S.P., Petroll, W.M., and Hulleman, J.D. Ω Fibulin-3 knockout mice demonstrate corneal dysfunction but maintain normal retinal integrity. Journal of Molecular Medicine, 98(11): 1639-1656 ( Ω -corresponding author) PMID: 32964303 |
Ramadurgum, P., Daniel, S., and Hulleman, J.D. Ω Protocol for in vivo evaluation and use of destabilizing domains in the eye, liver and beyond. Invited article. STAR Protocols, 1(2): 100094 ( Ω -corresponding author) PMID: 32995756 |
Rong, Z., Gong, X., Hulleman, J.D., Corey, D.R., and Mootha, V.V. (2020) Trinucleotide repeat-targeting dCas9 as a therapeutic strategy for Fuchs’ endothelial corneal dystrophy. Translation Vision Science and Technology, 9(9): 47 PMID: 32934897 |
Landeck, N., Strathearn, K.E., Ysselstein, D., Buck, K., Dutta, S., Banerjee, S., Lu, Z., Hulleman, J.D., Hindupur, J., Lin, L-K., Padalkar, S., McCabe, G.P., Stanciu, L.A., Lyubchenko, Y.L., Kirik, D., and Rochet, J-C. (2020) Two C-terminal sequence variations determine differential neurotoxicity between human and mouse α-synuclein. Molecular Neurodegeneration, 15(1): 49. PMID: 32900375 |
Ramadurgum, P., and Hulleman, J.D. Ω Protocol for designing small molecule-regulated destabilizing domains for in vitro use. Invited article. STAR Protocols, 1(2): 100069 ( Ω -corresponding author) PMID: 32995752 |
Zhou, M., Weber, S.R., Zhao, Y., Chen, H., Barber, A., Grillo, S.L., Wilis, C.A., Wang, H.G., Hulleman, J.D., and Sundstrom, J.M. (2020) Expression of R345W-Fibulin-3 induces epithelial-mesenchymal transition in retinal pigment epithelial cells. Frontiers in Cell and Developmental Biology, 8: 469 PMID: 32637411 |
Ramadurgum, P., Woodard, D.R., Daniel, S.N., Peng, H., Mallipeddi, P.L., Niederstrasser, H., Mihelakis, M., Chau, V.Q., Douglas, P.M., Posner, B.A., and Hulleman, J.D. Ω. Simultaneous control of endogenous and user-defined genetic pathways using new ecDHFR pharmacological chaperones. Cell Chemical Biology, 27(5): 622-634. ( Ω - corresponding author). PMID: 32330442 |
Chau, V.Q., Hu, J., Gong, X., Hulleman, J.D., Ufret-Vincenty, R.L., Rigo, F., Prakash, T.P., Corey, D., and Mootha, V.V. Delivery of antisense oligonucleotides to the corneal endothelium. Nucleic Acid Therapeutics, 30(4): 207-214. Featured Article in Nuc. Acid Ther. PMID: 32202944 |
2019
Datta, S., Peng, H., and Hulleman, J.D. Ω. Small molecule-based inducible gene therapies for retinal degeneration. Advances in Experimental Medicine and Biology, 1185: 65-69. ( Ω - corresponding author) PMID: 31884590 |
Peng, H.*, Chau, V.Q.*, Phetsang, W., Sebastian, R.L., Stone, M.R.L., Datta, S., Renwick, M., Tamer, Y.T., Toprak, E., Koh, A.Y., Blaskovich, M.A.T. and Hulleman, J.D. Ω. Non-antibiotic small-molecule regulation of DHFR-based destabilizing domains in vivo. Molecular Therapy – Methods and Clinical Development, 15: 27-39. (* - equal contribution, Ω - corresponding author) PMID: 31649953 |
Peng, H., and Hulleman, J.D. Ω. Prospective application of activity-based protein profiling in eye research – Insights into ocular protease biology and pathology. Invited review. International Journal of Molecular Science, 20(16): 3855. ( Ω - corresponding author) PMID: 31398819 |
2018
Datta, S., Renwick, M., Chau, V.Q., Zhang, F., Nettesheim, E., Lipinski, D.M., and Hulleman, J.D. Ω. A destabilizing domain allows for fast, non-invasive, conditional control of protein abundance in the mouse eye – Implications for ocular gene therapy*. 58(12): 4909-4920. Investigative Ophthalmology and Vision Science. ( Ω - corresponding author) *article chosen as a Featured Article, included in the Hot Topic 2017 Collection in IOVS and top 5 viewed paper of 2018 (Silverchair analytics). PMID: 30347085 |
Bamberger, C., MartínezBartolomé, S., Montgomery, M., Pankow, S. Hulleman, J.D., Kelly, J.W., and Yates, J.R. Deducing missing proteins and proteoforms in quantitative proteomics. Nature Communications, 9(1): 2320 PMID: 29899466 |
Wong, M.Y., Doan, N.D., DiChiara, Papa, L., A.S., Cheah, J.H., Soule, C.K., Watson, N., Hulleman, J.D., and Shoulders, M.D. A high-throughput assay for collagen secretion suggests an unanticipated role for Hsp90 in collagen production. Biochemistry, 57(19): 2814-2827. PMID: 29676157 |
2017
Vu, K.T., Zhang, F., and Hulleman, J.D. Ω Conditional, genetically-encoded, small molecule-regulated inhibition of NFB signaling in RPE cells*. Investigative Ophthalmology and Vision Science, 58(10): 4126-4137 ( Ω - corresponding author). *article chosen as a Featured Article, Most Viewed and an Editor’s Top Pick. PMID: 28829844 |
Vu, K.T., and Hulleman, J.D. Ω An inducible form of Nrf2 confers enhanced protection against acute oxidative stresses in RPE cells. Experimental Eye Research, 164: 31-36 ( Ω - corresponding author). PMID: 28782506 |
2016
Hulleman, J.D. *Ω , Genereux, J.C. * , and Nguyen, A. Mapping wild-type and R345W fibulin-3 intracellular interactomes. Experimental Eye Research, 153: 165-169 (* - equal contribution, Ω - corresponding author). PMID: 27777122 |
Zadoo, S., Nguyen, A., Zode, G., and Hulleman, J.D. Ω A novel luciferase assay for sensitively monitoring myocilin variants in cell culture. Investigative Ophthalmology and Vision Science 57(4): 1939-1950 ( Ω - corresponding author). PMID: 27092720 |
Nguyen, A. and Hulleman, J.D. Ω Evidence of alternative cystatin c signal sequence cleavage which is influenced by the A25T polymorphism. PLoS One. 11(2) ( Ω - corresponding author). PMID: 26845025 |
Hulleman, J.D., Nguyen, A., Ramprasad, V.L., Murugan, S., Gupta, R., Mahindrakar, A., Angara, R., Sankurathri, C., and Mootha, V.V. (2016) A novel H395R mutation in MKKS/BBS6 causes retinitis pigmentosa and polydactyly without other findings of Bardet-Biedl or McKusick-Kaufman syndrome. Molecular Vision, 22: 73-81. PMID: 26900326 |
Hulleman, J.D. Ω Malattia Leventinese/Doyne Honeycomb Retinal Dystrophy: Similarities to age-related macular degeneration and potential therapies. Advances in Experimental Medicine and Biology, 854: 153-158. ( Ω - corresponding author). PMID: 26427406 |
2015
Nguyen, A. and Hulleman, J.D. Ω Differential tolerance of ‘pseudo-pathogenic’ tryptophan residues in calcium- binding EGF domains of short fibulin proteins. Experimental Eye Research, 130: 66-72. ( Ω - corresponding author). PMID: 25481286 |
Hulleman, J.D. # and Kelly, J.W. # (2015) Genetic ablation of N-linked glycosylation reveals two important folding pathways for mutant fibulin-3, a protein associated with retinal degeneration. FASEB Journal, 29(2): 565-75 ( # - co- corresponding author). PMID: 25389134 |
2014
Chen, J.J., Genereux, J.G., Qu, S., Hulleman, J.D., Shoulders, M.D., and Wiseman, R.L. Increasing ER quality control stringency through ATF6 activation reduces the secretion and extracellular aggregation of destabilized variants of an amyloidogenic protein. Chemistry and Biology 21(11): 1–11. PMID: 25444553 |
Cooley, C.B., Ryno, L.M., Plate, L., Morgan, G.J., Hulleman, J.D., Kelly, J.W., and Wiseman, R.L. Unfolded protein response activation reduces secretion and extracellular aggregation of amyloidogenic immunoglobulin light chain. Proceedings of the National Academy of Science, U.S.A. 111(36): 13046-13051. PMID: 25157167 |
Wang, X., Cattaneo, F., Ryno, L.M., Hulleman, J.D., Reixach, N., and Buxbaum, J.N. (2014) 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. Journal of Neuroscience, 34(21): 7253-65. PMID: 24849358 |
2013
Hulleman, J.D. # , Brown, S.J., Rosen, H., and Kelly, J.W. # A high-throughput cell-based Gaussia luciferase reporter assay for identifying modulators of fibulin-3 secretion. Journal of Biomolecular Screening, 18(6): 647-658 ( # - co- corresponding author). PMID: 23230284 |
2012
Hulleman, J.D. # , Balch, W.E., and Kelly, J.W. # Translational attenuation differentially alters the fate of disease- associated fibulin proteins. FASEB Journal, 26 (11): 4548-4560 ( # - co-corresponding author). PMID: 22872678 |
Madian, A.G., Hindupur, J., Hulleman, J.D., Diaz-Maldonado, N., Guigard, E., Kay, C.M., Rochet, J-C., and Regnier, F.E. Effect of single amino acid substitution on oxidative modifications of the Parkinson’s disease-related protein, DJ-1. Molecular & Cellular Proteomics, 11 (2): M111.010892. PMID: 22104028 |
2011
Hulleman, J.D.# , Kaushal, S., Balch, W.E., and Kelly, J.W. # Compromised mutant EFEMP1 secretion remedied by proteostasis network alteration. Molecular Biology of the Cell, 22 (24): 4765-4775 ( # - co-corresponding author). PMID: 22031286 |
2009
Usui, K.*, Hulleman, J.D.*, Paulsson, J.F., Siegel, S.S., Powers, E.T., and Kelly, J.W. Site-specific modification of Alzheimer's peptides by cholesterol oxidation products enhances aggregation energetics and neurotoxicity. Proceedings of the National Academy of Science, U.S.A., 106 (44): 18563-18568 (* - equal contribution). PMID: 19841277 |
Padalkar, S., Hulleman, J.D., Kim, S.M., Tumkur, T., Rochet, J-C., Stach, E., and Stanciu, L.A. Fabrication of ZnS nanoparticle chains on a protein template. Journal of Nanoparticle Research (doi: 10.1007/s11051-009-9689-8 ). PMID: 21804765 |
Rochet, J-C. and Hulleman, J.D. α-Synuclein self-assembly in Parkinson’s disease: insights from biophysical analyses. In T.F. Outeiro (Ed.), Protein Misfolding in Biology and Disease. Trivandrum, India: Research Signpost. |
2008
Liu, F., Nguyen, J.L., Hulleman, J.D., Li, L., Rochet, J-C. Neuroprotective function of DJ-1 and MsrA in a cellular model of Parkinson’s disease. Journal of Neurochemistry, 105 (6): 2435-2453. PMID: 18331584 |
Padalkar, S., Hulleman, J.D., Rochet, J-C., Stach, E., Stanciu, L. Protein-templated semiconductor nanoparticle chains. Nanotechnology, 19 (27) (doi: 10.1088/0957-4484/19/27/275602 ). PMID: 21828709 |
Colby, R., Hulleman, J.D., Padalkar, S., Rochet, J-C., Stanciu, L. Biotemplated synthesis of metallic nanoparticle chains on an α-synuclein fiber scaffold. Journal of Nanoscience and Nanotechnology, 8 (2): 973–978. PMID: 18464436 |
2007
Zakharov, S., Hulleman, J.D., Dutseva, E., Antonenko, Y., Rochet, J-C., Cramer, W.A. (2007) Helical α-synuclein forms highly conductive ion channels. Biochemistry, 46 (50): 14369-14379. PMID: 18031063 |
Hulleman, J.D., Mirzaei, H., Guigard, E., Taylor, K.L., Ray, S.S., Kay, C.M., Regnier, F.E., Rochet, J-C. Destabilization of DJ-1 by a familial substitution and oxidative modifications: implications for Parkinson’s disease. Biochemistry, 46 (19): 5776-5789. PMID: 17451229 |
Padalkar, S., Hulleman, J.D., Colby, R., Cunzeman, K., Rochet, J-C., Stach, E., Stanciu, L. (2007) α-synuclein as a template for the synthesis of metallic nanowires. Nanotechnology, 18 (5) (doi: 10.1088/0957- 4484/18/5/055609 ). |