Collaborative Publications | Conformational Protein Diseases Center

The Center for Protein Conformational Diseases is composed of a group of highly collaborative investigators whose scientific interests overlap. The breadth and number of collaborative publications amongst members of this group highlight this fact, as well as the trans-departmental and multi-disciplinary approaches used to understand how misfolded proteins lead to human disease.

2023

Boyd-Shiwarski CR, Shiwarski DJ, Griffiths SE, Beacham RT, Norrell L, Morrison DE, Wang J, Mann J, Tennant W, Anderson EN, Franks J, Calderon M, Connolly KA, Cheema MU, Weaver CJ, Nkashama LJ, Weckerly CC, Querry KE, Pandey UB, Donnelly CJ, Sun D, Rodan AR, Subramanya AR. WNK kinases sense molecular crowding and rescue cell volume via phase separation. Cell. 2022 Nov 23;185(24):4488-4506.e20.

Nguyen NH, Sarangi S, McChesney EM, Sheng S, Durrant JD, Porter AW, Kleyman TR, Pitluk ZW, Brodsky JL. Genome mining yields putative disease-associated ROMK variants with distinct defects. PLoS Genet. 2023 Nov 13;19(11):e1011051.

Van Laar AD, Webb KR, Keeney MT, Van Laar VS, Zharikov A, Burton EA, Hastings TG, Glajch KE, Hirst WD, Greenamyre JT, Rocha EM. Transient exposure to rotenone causes degeneration and progressive parkinsonian motor deficits, neuroinflammation, and synucleinopathy. NPJ Parkinsons Dis. 2023 Aug 11;9(1):121.

DeGiosio RA, Needham PG, Andrews OA, Tristan H, Grubisha MJ, Brodsky JL, Camacho C, Sweet RA. Differential regulation of MAP2 by phosphorylation events in proline-rich versus C-terminal domains. FASEB J. 2023 Oct;37(10):e23194.

Sukhoplyasova M, Keith AM, Perrault EM, Vorndran HE, Jordahl AS, Yates ME, Pastor A, Li Z, Freaney ML, Deshpande RA, Adams DB, Guerriero CJ, Shi S, Kleyman TR, Kashlan OB, Brodsky JL, Buck TM. Lhs1 dependent ERAD is determined by transmembrane domain context. Biochem J. 2023 Sep 27;480(18):1459-1473

Bhatia TN, Jamenis AS, Abbas M, Clark RN, Miner KM, Chandwani MN, Kim RE, Hilinski W, O'Donnell LA, Luk KC, Shi Y, Hu X, Chen J, Brodsky JL, Leak RK. A 14-day pulse of PLX5622 modifies α-synucleinopathy in preformed fibril-infused aged mice of both sexes. Neurobiol Dis. 2023 Aug;184:106196

Lesko MA, Chandrashekarappa DG, Jordahl EM, Oppenheimer KG, Bowman RW 2nd, Shang C, Durrant JD, Schmidt MC, O'Donnell AF. Changing course: Glucose starvation drives nuclear accumulation of Hexokinase 2 in S. cerevisiae. PLoS Genet. 2023 May 17;19(5):e1010745.

Sannino S, Manuel AM, Shang C, Wendell SG, Wipf P, Brodsky JL. Non-Essential Amino Acid Availability Influences Proteostasis and Breast Cancer Cell Survival During Proteotoxic Stress. Mol Cancer Res. 2023 Jul 5;21(7):675-690.

2022

Shi S, Buck TM, Nickerson AJ, Brodsky JL, Kleyman TR. Paraoxonase 2 is an ER chaperone that regulates the epithelial Na+ channel. Am J Physiol Cell Physiol. In press

Bhatia TN, Clark RN, Needham PG, Miner KM, Jamenis AS, Eckhoff EA, Abraham N, Hu X, Wipf P, Luk KC, Brodsky JL, Leak RK. Heat Shock Protein 70 as a Sex-Skewed Regulator of α-Synucleinopathy. Neurotherapeutics. In press

Todd NK, Huang Y, Lee JY, Doruker P, Krieger JM, Salisbury R, MacDonald M, Bahar I, Thathiah A. GPCR kinases generate an APH1A phosphorylation barcode to regulate amyloid-β generation. Cell Rep. 2022 Jul 19;40(3):111110.

Porter AW, Nguyen DN, Clayton DR, Ruiz WG, Mutchler SM, Ray EC, Marciszyn AL, Nkashama LJ, Subramanya AR, Gingras S, Kleyman TR, Apodaca G, Hendershot LM, Brodsky JL, Buck TM. The molecular chaperone GRP170 protects against ER stress and acute kidney injury in mice. JCI Insight. 2022 Mar 8;7(5):e151869

Keeney MT, Hoffman EK, Farmer K, Bodle CR, Fazzari M, Zharikov A, Castro SL, Hu X, Mortimer A, Kofler JK, Cifuentes-Pagano E, Pagano PJ, Burton EA, Hastings TG, Greenamyre JT, Di Maio R. NADPH oxidase 2 activity in Parkinson's disease. Neurobiol Dis. 2022 Aug;170:105754.

Montalbetti N, Przepiorski AJ, Shi S, Sheng S, Baty CJ, Maggiore JC, Carattino MD, Vanichapol T, Davidson AJ, Hukriede NA, Kleyman TR. Functional characterization of ion channels expressed in kidney organoids derived from human induced pluripotent stem cells. Am J Physiol Renal Physiol. 2022 Oct 1;323(4):F479-F491.

Przepiorski A, Vanichapol T, Espiritu EB, Crunk AE, Parasky E, McDaniels MD, Emlet DR, Salisbury R, Happ CL, Vernetti LA, MacDonald ML, Kellum JA, Kleyman TR, Baty CJ, Davidson AJ, Hukriede NA. Modeling oxidative injury response in human kidney organoid. Stem Cell Res Ther. 2022 Feb 21;13(1):76.

2021

Sorkina T, Cheng MH, Bagalkot TR, Wallace C, Watkins SC, Bahar I, Sorkin A. Direct coupling of oligomerization and oligomerization-driven endocytosis of the dopamine transporter to its conformational mechanics and activity. J Biol Chem. 2021; 296:100430.

Goeckeler-Fried JL, Aldrin Denny R, Joshi D, Hill C, Larsen MB, Chiang AN, Frizzell RA, Wipf P, Sorscher EJ, Brodsky JL. Improved correction of F508del-CFTR biogenesis with a folding facilitator and an inhibitor of protein ubiquitination. Bioorg Med Chem Lett. 2021; 48:128243.

Sannino S, Yates ME, Schurdak ME, Oesterreich S, Lee AV, Wipf P, Brodsky JL. Unique integrated stress response sensors regulate cancer cell susceptibility when Hsp70 activity is compromised. Elife. 2021; 10:e64977.

Rastelli EJ, Sannino S, Hart DJ, Sharlow ER, Lazo JS, Brodsky JL, Wipf P. Synthesis and evaluation of bifunctional PTP4A3 phosphatase inhibitors activating the ER stress pathway. Bioorg Med Chem Lett. 2021; 46:128167.

Sun WY, Tyurin VA, Mikulska-Ruminska K, Shrivastava IH, Anthonymuthu TS, Zhai YJ, Pan MH, Gong HB, Lu DH, Sun J, Duan WJ, Korolev S, Abramov AY, Angelova PR, Miller I, Beharier O, Mao GW, Dar HH, Kapralov AA, Amoscato AA, Hastings TG, Greenamyre TJ, Chu CT, Sadovsky Y, Bahar I, Bayır H, Tyurina YY, He RR, Kagan VE. Phospholipase iPLA2β averts ferroptosis by eliminating a redox lipid death signal. Nat Chem Biol. 2021; 17(4):465-476.

Ray EC, Carrisoza-Gaytan R, Al-Bataineh M, Marciszyn AL, Nkashama LJ, Chen J, Winfrey A, Griffiths S, Lam TR, Flores D, Wu P, Wang W, Huang CL, Subramanya AR, Kleyman TR, Satlin LM. L-WNK1 is required for BK channel activation in intercalated cells. Am J Physiol Renal Physiol. 2021; 321(2):F245-F254.

Fortuna TR, Kour S, Anderson EN, Ward C, Rajasundaram D, Donnelly CJ, Hermann A, Wyne H, Shewmaker F, Pandey UB. DDX17 is involved in DNA damage repair and modifies FUS toxicity in an RGG-domain dependent manner. Acta Neuropathol. 2021; 142(3):515-536.

Anderson EN, Morera AA, Kour S, Cherry JD, Ramesh N, Gleixner A, Schwartz JC, Ebmeier C, Old W, Donnelly CJ, Cheng JP, Kline AE, Kofler J, Stein TD, Pandey UB. Traumatic injury compromises nucleocytoplasmic transport and leads to TDP-43 pathology. Elife. 2021; 10:e67587.

2020

Ponzoni L, Nguyen NH, Bahar I, Brodsky JL. Complementary computational and experimental evaluation of missense variants in the ROMK potassium channel. PLoS Comput Biol. 2020 Apr 6;16(4):e1007749.

Thermozier S, Hou W, Zhang X, Shields D, Fisher R, Bayir H, Kagan V, Yu J, Liu B, Bahar I, Epperly MW, Wipf P, Wang H, Huq MS, Greenberger JS. Anti-Ferroptosis Drug Enhances Total-Body Irradiation Mitigation by Drugs that Block Apoptosis and Necroptosis. Radiat Res. 2020 May;193(5):435-450.

Singh A, Vashistha N, Heck J, Tang X, Wipf P, Brodsky JL, Hampton RY. Direct involvement of Hsp70 ATP hydrolysis in Ubr1-dependent quality control. Mol Biol Cell. 2020 Nov 15;31(24):2669-2686.

Terrab L, Rosenker CJ, Johnstone L, Ngo LK, Zhang L, Ware NF, Miller B, Topacio AZ, Sannino S, Brodsky JL, Wipf P. Synthesis and Selective Functionalization of Thiadiazine 1,1-Dioxides with Efficacy in a Model of Huntington's Disease. ACS Med Chem Lett. 2020 Feb 20;11(5):984-990.

Van Laar VS, Chen J, Zharikov AD, Bai Q, Di Maio R, Dukes AA, Hastings TG, Watkins SC, Greenamyre JT, St Croix CM, Burton EA. α-Synuclein amplifies cytoplasmic peroxide flux and oxidative stress provoked by mitochondrial inhibitors in CNS dopaminergic neurons in vivo. Redox Biol. 2020 Aug 22;37:101695.

Ramesh N, Daley EL, Gleixner AM, Mann JR, Kour S, Mawrie D, Anderson EN, Kofler J, Donnelly CJ, Kiskinis E, Pandey UB. RNA dependent suppression of C9orf72 ALS/FTD associated neurodegeneration by Matrin-3. Acta Neuropathol Commun. 2020 Oct 31;8(1):177.

Otte CG, Fortuna TR, Mann JR, Gleixner AM, Ramesh N, Pyles NJ, Pandey UB, Donnelly CJ. Optogenetic TDP-43 nucleation induces persistent insoluble species and progressive motor dysfunction in vivo. Neurobiol Dis. 2020 Dec;146:105078.

Carrisoza-Gaytan R, Ray EC, Flores D, Marciszyn AL, Wu P, Liu L, Subramanya AR, Wang W, Sheng S, Nkashama LJ, Chen J, Jackson EK, Mutchler SM, Heja S, Kohan DE, Satlin LM, Kleyman TR. Intercalated cell BKα subunit is required for flow-induced K+ secretion. JCI Insight. 2020 Apr 7;5(8):e130553.

2019

Chiang AN, Liang M, Dominguez-Meijide A, Masaracchia C, Goeckeler-Fried JL, Mazzone CS, Newhouse DW, Kendsersky NM, Yates ME, Manos-Turvey A, Needham PG, Outeiro TF, Wipf P, Brodsky JL. Synthesis and evaluation of esterified Hsp70 agonists in cellular models of protein aggregation and folding. Bioorg Med Chem. 2019 27(1):79-91.

Mann JR, Gleixner AM, Mauna JC, Gomes E, DeChellis-Marks MR, Needham PG, Copley KE, Hurtle B, Portz B, Pyles NJ, Guo L, Calder CB, Wills ZP, Pandey UB,Kofler JK, Brodsky JL, Thathiah A, Shorter J, Donnelly CJ. RNA Binding Antagonizes Neurotoxic Phase Transitions of TDP-43. Neuron. 2019 102(2):321-338.

Cheng MH, Ponzoni L, Sorkina T, Lee JY, Zhang S, Sorkin A, Bahar I. Trimerization of dopamine transporter triggered by AIM-100 binding: Molecular mechanism and effect of mutations. Neuropharmacology. 2019 161:107676.

Zharikov A, Bai Q, De Miranda BR, Van Laar A, Greenamyre JT, Burton EA. Long-term RNAi knockdown of α synuclein in the adult rat substantia nigra without neurodegeneration. Neurobiol Dis. 2019 125:146-153.

2018

Boyd-Shiwarski CR, Shiwarski DJ, Roy A, Namboodiri HN, Nkashama LJ, Xie J, McClain KL, Marciszyn A, Kleyman TR, Tan RJ, Stolz DB, Puthenveedu MA, Huang CL, Subramanya AR. Potassium-regulated distal tubule WNK bodies are kidney-specific WNK1 dependent. Mol Biol Cell. 2018 29(4):499-509.

De Miranda BR, Rocha EM, Bai Q, El Ayadi A, Hinkle D, Burton EA, Greenamyre JT. Astrocyte-specific DJ-1 overexpression protects against rotenone-induced neurotoxicity in a rat model of Parkinson's disease. Neurobiol Dis. 2018 115:101-114.

Di Maio R, Hoffman EK, Rocha EM, Keeney MT, Sanders LH, De Miranda BR, Zharikov A, Van Laar A, Stepan AF, Lanz TA, Kofler JK, Burton EA, Alessi DR, Hastings TG, Greenamyre JT. LRRK2 activation in idiopathic Parkinson's disease. Sci Transl Med. 2018 10(451).

Hager NA, Krasowski CJ, Mackie TD, Kolb AR, Needham PG, Augustine AA, Dempsey A, Szent-Gyorgyi C, Bruchez MP, Bain DJ, Kwiatkowski AV, O'Donnell AF, Brodsky JL. Select α-arrestins control cell-surface abundance of the mammalian Kir2.1 potassium channel in a yeast model. J Biol Chem. 2018 293(28):11006-11021.

Kashlan OB, Kinlough CL, Myerburg MM, Shi S, Chen J, Blobner BM, Buck TM, Brodsky JL, Hughey RP, Kleyman TR. N-linked glycans are required on epithelial Na(+) channel subunits for maturation and surface expression. Am J Physiol Renal Physiol. 2018 314(3):F483-F492.

Kaya C, Cheng MH, Block ER, Bartol TM, Sejnowski TJ, Sorkin A, Faeder JR, Bahar I. Heterogeneities in Axonal Structure and Transporter Distribution Lower Dopamine Reuptake Efficiency. eNeuro. 2018 5(1). pii: ENEURO.0298-17.2017.

Mackie TD, Kim BY, Subramanya AR, Bain DJ, O'Donnell AF, Welling PA, Brodsky JL. The endosomal trafficking factors CORVET and ESCRT suppress plasma membrane residence of the renal outer medullary potassium channel (ROMK). J Biol Chem. 2018 293(9):3201-3217.

Sannino S, Guerriero CJ, Sabnis AJ, Stolz DB, Wallace CT, Wipf P, Watkins SC, Bivona TG, Brodsky JL. Compensatory increases of select proteostasis networks after Hsp70 inhibition in cancer cells. J Cell Sci. 2018 131(17). pii: jcs217760.

Sheng S, Chen J, Mukherjee A, Yates ME, Buck TM, Brodsky JL, Tolino MA, Hughey RP, Kleyman TR. Thumb domains of the three epithelial Na(+) channel subunits have distinct functions. J Biol Chem. 2018 293(45):17582-17592.

Welty S, Teng Y, Liang Z, Zhao W, Sanders LH, Greenamyre JT, Rubio ME, Thathiah A, Kodali R, Wetzel R, Levine AS, Lan L. RAD52 is required for RNA-templated recombination repair in post-mitotic neurons. J Biol Chem. 2018 293(4):1353-1362.

2017

Boatz JC, Whitley MJ, Li M, Gronenborn AM, and Van der Wel PCA. Cataract-associated P23T γD-crystallin retains a native-like fold in amorphous-looking aggregates formed at physiological pH. Nat Commun. 2017; 8:15137

Buck TM, Jordahl AS, Yates ME, Preston GM, Cook E, Kleyman TR, Brodsky JL. Interactions between intersubunit transmembrane domains regulate the chaperone-dependent degradation of an oligomeric membrane protein. Biochem J. 2017;474(3):357-376.

Lin HK, Boatz JC, Krabbendam IE, Kodali R, Hou Z, Wetzel R, Dolga AM, Poirier MA, van der Wel PCA. Fibril polymorphism affects immobilized non-amyloid flanking domains of huntingtin exon1 rather than its polyglutamine core. Nat Commun. 2017; 8:15462

O’Donnell, B.M., T.D. Mackie, A.R. Subramanya, and J.L. Brodsky. Endoplasmic Reticulum Associated Degradation of the Renal Outer Medullary Potassium Channel, ROMK, Underlies Type II Bartter Syndrome. J. Biol. Chem. (2017), In press

Kar, K., Baker, M.A., Lengyel, G.A., Hoop, C.L., Kodali, R., Byeon, I.J., Horne, W.S., van der Wel, P.C.A., & Wetzel, R., Backbone engineering within a latent β-hairpin structure to design inhibitors of polyglutamine amyloid formation. J. Mol. Biol. 2017; 429: 308-323

Boyd-Shiwarski CR, Shiwarski DJ, Roy A, Nkashama LJ, Namboodiri HN, Xie J, McClain KL, Marciszyn A, Kleyman TR, Tan RJ, Stolz DB, Puthenveedu MA, Huang CL, Subramanya AR. Potassium-Regulated Distal Tubule WNK Bodies are Kidney-Specific WNK1 Dependent. Mol Biol Cell. 2017; in press

Steinman J, Epperly M, Hou W, Willis J, Wang H, Fisher R, Liu B, Bahar I, McCaw T, Kagan V, Bayir H, Yu J, Wipf P, Li S, Huq MS, Greenberger JS. Improved Total-Body Irradiation Survival by Delivery of Two Radiation Mitigators that Target Distinct Cell Death Pathways. Radiat Res. 2017; in press

Kashlan OB, Kinlough CL, Myerburg MM, Shi S, Chen J, Blobner BM, Buck TM, Brodsky JL, Hughey RP, Kleyman TR. N-linked Glycans are Required on Epithelial Na+; Channel Subunits for Maturation and Surface Expression. Am J Physiol Renal Physiol. 2017; in press

Welty S, Teng Y, Liang Z, Zhao W, Sanders L, Greenamyre JT, Rubio ME, Thathiah A, Kodali R, Wetzel R, Levine AS, Lan L. RAD52 is required for RNA-templated recombination repair in post-mitotic neurons. J Biol Chem. 2017; in press

Ma S, Cheng MH, Guthrie DA, Newman AH, Bahar I, Sorkin A. Targeting of dopamine transporter to filopodia requires an outward-facing conformation of the transporter. Sci Rep. 2017; 7(1):5399.

Ye Z, Needham PG, Estabrooks SK, Whitaker SK, Garcia BL, Misra S, Brodsky JL, Camacho CJ. Symmetry breaking during homodimeric assembly activates an E3 ubiquitin ligase. Sci Rep. 2017; 7(1):1789.

Gleixner AM, Hutchison DF, Sannino S, Bhatia TN, Leak LC, Flaherty PT, Wipf P, Brodsky JL, Leak RK. N-Acetyl-l-Cysteine Protects Astrocytes against Proteotoxicity without Recourse to Glutathione. Mol Pharmacol. 2017; 92(5):564-575.

Shi S, Buck TM, Kinlough CL, Marciszyn AL, Hughey RP, Chalfie M, Brodsky JL, Kleyman TR. Regulation of the epithelial Na+; channel by paraoxonase-2. J Biol Chem. 2017; 292(38):15927-15938.

2016

Di Maio, R., PJ Barrett, EK Hoffman, CW Barrett, A Zharikov, A Borah, X Hu, J McCoy, CT Chu, EA Burton, TG Hastings, JT Greenamyre. a-Synuclein binds TOM20 and inhibits mitochondrial protein import in Parkinson’s disease. Sci Transl Med 2016; 8: 342ra78

Manos-Turvey A, Al-Ashtal HA, Needham PG, Hartline CB, Prichard MN, Wipf P, Brodsky JL. Dihydropyrimidinones and -thiones with improved activity against human polyomavirus family members. Bioorg Med Chem Lett. 2016;26(20):5087-5091

Sabnis AJ, Guerriero CJ, Olivas V, Sayana A, Shue J, Flanagan J, Asthana S, Paton AW, Paton JC, Gestwicki JE, Walter P, Weissman JS, Wipf P, Brodsky JL, Bivona TG. Combined chemical-genetic approach identifies cytosolic HSP70 dependence in rhabdomyosarcoma. Proc Natl Acad Sci U S A. 2016;113(32):9015-20

Hoop, C.L., Lin, H.-K., Kar, K., Magyarfalvi, G., Lamley, J.M., Boatz, J.C., Mandal, A., Lewandowski, J.R., Wetzel, R., & van der Wel, P.C.A., Huntingtin exon 1 fibrils feature an interdigitated β-hairpin-based polyglutamine core. Proc. Natl. Acad. Sci. USA. 2016; 113: 1546-1551

Sahoo, B., Arduini, I., Drombosky, K.W., Kodali, R., Saunders, L.H., Greenamyre, J.T., & Wetzel, R., Folding landscape of mutant Huntingtin exon1: Diffusible multimers, oligomers and fibrils, and no detectable monomer. PLoS One. 2016; DOI: 10.1371/journal.pone.0155747

Webb TN, Carrisoza-Gaytan R, Montalbetti N, Rued A, Roy A, Socovich AM, Subramanya AR, Satlin LM, Kleyman TR, Carattino MD. Cell-specific regulation of L-WNK1 by dietary K. Am J Physiol Renal Physiol. 2016;310(1):F15-26.

Veit G, Avramescu RG, Chiang AN, Houck SA, Cai Z, Peters KW, Hong JS, Pollard HB, Guggino WB, Balch WE, Skach WR, Cutting GR, Frizzell RA, Sheppard DN, Cyr DM, Sorscher EJ, Brodsky JL, Lukacs GL. From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations. Mol Biol Cell. 2016;27(3):424-33

2015

Brodsky JL, Frizzell RA. A combination therapy for cystic fibrosis. Cell. 2015;163(1):17

Buck TM, Jordan R, Lyons-Weiler J, Adelman JL, Needham PG, Kleyman TR, Brodsky JL. Expression of three topologically distinct membrane proteins elicits unique stress response pathways in the yeast Saccharomyces cerevisiae. Physiol Genomics. 2015;47(6):198-214

Zharikov A, Cannon JR, Tapias V, Bai Q, Horowitz M, Shah V, El Ayadi A, Hastings TG, Greenamyre JT, Burton EA, shRNA targeting α-synuclein prevents neurodegeneration in a Parkinson’s disease model, J Clin Invest 2015; 125:2721-35

Wipf P, Eyer BR, Yamaguchi Y, Zhang F, Neal MD, Sodhi CP, Good M, Branca M, Prindle T Jr, Lu P, Brodsky JL, Hackam DJ. Synthesis of anti-inflammatory α-andβ-linked acetamidopyranosides as inhibitors of toll-like receptor 4 (TLR4. Tetrahedron Lett. 2015;56(23):3097-3100

Posimo JM, Weilnau JN, Gleixner AM, Broeren MT, Weiland NL, Brodsky JL, Wipf P, Leak RK. Heat shock protein defenses in the neocortex and allocortex of thetelencephalon. Neurobiol Aging. 2015 May;36(5):1924-37

Crum TS, Gleixner AM, Posimo JM, Mason DM, Broeren MT, Heinemann SD, Wipf P, Brodsky JL, Leak RK. Heat shock protein responses to aging and proteotoxicity in the olfactory bulb. J Neurochem. 2015;133(6):780-94

Needham PG, Patel HJ, Chiosis G, Thibodeau PH, Brodsky JL. Mutations in the Yeast Hsp70, Ssa1, at P417 Alter ATP Cycling, Interdomain Coupling, and Specific Chaperone Functions. J Mol Biol. 2015;427(18):2948-65

Cheng MH, Block E, Hu F, Cobanoglu MC, Sorkin A, Bahar I. Insights into the modulation of dopamine transporter function by amphetamine, orphenadrine, and cocaine binding. Front Neurol 2015; 6: 134

Chen J, Ray EC, Yates ME, Buck TM, Brodsky JL, Kinlough CL, Winarski KL, Hughey RP, Kleyman TR, Sheng S. Functional Roles of Clusters of Hydrophobic and Polar Residues in the Epithelial Na+ Channel Knuckle Domain. J Biol Chem. 2015;290(41):25140-50

Prosser DC, Pannunzio AE, Brodsky JL, Thorner J, Wendland B, O'Donnell AF. α-Arrestins participate in cargo selection for both clathrin-independent and clathrin-mediated endocytosis. J Cell Sci. 2015;128(22):4220-34

2014

Kar, K., Arduini, I., Drombosky, K.W., van der Wel, P.C.A., & Wetzel, R., d-polyglutamine amyloid recruits l-polyglutamine monomers and kills cells, J. Mol. Biol. 2014; 426: 816-829

Hoop, C., Lin, H.-K., Kar, K., Hou, Z., Poirier, M., Wetzel, R., & van der Wel, P.C.A., Polyglutamine amyloid core boundaries and flanking domain dynamics in huntingtin fragment fibrils determined by solid-state NMR. Biochem. 2014; 53: 6653-6666

Hecht KA, O'Donnell AF, Brodsky JL. The proteolytic landscape of the yeast vacuole. Cell Logist. 2014;4(1):e28023

LH Sanders, J McCoy, X Hu, PG Mastroberardino, BC Dickinson, CJ Chang, CT Chu, B Van Houten, JT Greenamyre. Mitochondrial DNA damage: molecular marker of vulnerable nigral neurons in Parkinson’s disease. Neurobiol Dis 2014; 70: 214-223

Alvaro CG, O'Donnell AF, Prosser DC, Augustine AA, Goldman A, Brodsky JL, Cyert MS, Wendland B, Thorner J. Specific α-arrestins negatively regulate Saccharomyces cerevisiae pheromone response by down-modulating the G-protein-coupled receptor Ste2. Mol Cell Biol. 2014;34(14):2660-81

Ireland AW, Gobillot TA, Gupta T, Seguin SP, Liang M, Resnick L, Goldberg MT, Manos-Turvey A, Pipas JM, Wipf P, Brodsky JL. Synthesis and structure–activity relationships of small molecule inhibitors of the simian virus 40 T antigen oncoprotein, an anti-polyomaviral target. Bioorg Med Chem. 2014;22(22):6490-502

Adam C, Baeurle A, Brodsky JL, Wipf P, Schrama D, Becker JC, Houben R. The HSP70 modulator MAL3-101 inhibits Merkel cell carcinoma. PLoS One 2014;9(4):e92041

2013

Wang Z, Subramanya AR, Satlin LM, Pastor-Soler NM, Carattino MD, Kleyman TR. Regulation of large-conductance Ca2+-activated K+ channels by WNK4 kinase. Am J Physiol Cell Physiol. 2013;305(8):C846-53.

Neal MD, Jia H, Eyer B, Good M, Guerriero CJ, Sodhi CP, Afrazi A, Prindle T Jr, Ma C, Branca M, Ozolek J, Brodsky JL, Wipf P, Hackam DJ. Discovery and validation of a new class of small molecule Toll-like receptor 4 (TLR4) inhibitors. PLoS One. 2013;8(6):e65779

CT Chu, J Ji, RK Dagda, JF Jiang, YY Tyurina, AA Kapralov, VA Tyurin, N Yanamala, IH Shrivastava, D Mohammadyani, KZQ Wang, J Zhu, J Klein-Seetharaman, K Balasubramanian, AA Amoscato, G Borisenko, Z Huang, AM Gusdon, A Cheikhi, EK Steer, R Wang, C Baty, S Watkins, I Bahar, H Bayır & VE Kagan Cardiolipin externalization to the outer mitochondrial membrane acts as an elimination signal for mitophagy in neuronal cells. Nature Cell Biol 2013; 15:1197-1205

Buck TM, Plavchak L, Roy A, Donnelly BF, Kashlan OB, Kleyman TR, Subramanya AR, Brodsky JL. The Lhs1/GRP170 chaperones facilitate the endoplasmic reticulum-associated degradation of the epithelial sodium channel. J Biol Chem. 2013;288(25):18366-80

Kar, K., Hoop, C.L., Drombosky, K.W., Baker, M.A., Kodali, R., Arduini, I., van der Wel, P.C.A., Horne, W.S., & Wetzel, R., b-hairpin-mediated nucleation of polyglutamine amyloid formation. J. Mol. Biol. 2013; 425: 1183-1197

Donnelly BF, Needham PG, Snyder AC, Roy A, Khadem S, Brodsky JL, Subramanya AR. Hsp70 and Hsp90 multichaperone complexes sequentially regulate thiazide-sensitive cotransporter endoplasmic reticulum-associated degradation and biogenesis. J Biol Chem. 2013;288(18):13124-35

Kilpatrick K, Novoa JA, Hancock T, Guerriero CJ, Wipf P, Brodsky JL, Segatori L. Chemical induction of Hsp70 reduces α-synuclein aggregation in neuroglioma cells. ACS Chem Biol. 2013;8(7):1460-8

2012

Seguin SP, Ireland AW, Gupta T, Wright CM, Miyata Y, Wipf P, Pipas JM, Gestwicki JE, Brodsky JL. A screen for modulators of large T antigen's ATPase activity uncovers novel inhibitors of Simian Virus 40 and BK virus replication. Antiviral Res. 2012;96(1):70-81

Jayaraman, M., Mishra, R., Kodali, R., Thakur, A.K., Koharudin, L.M.I., Gronenborn, A.M., & Wetzel, R., Kinetically competing huntingtin aggregation pathways control amyloid polymorphism and properties. Biochem. 2012; 51: 2706-2716

Liu L, Byeon IJ, Bahar I, Gronenborn AM. Domain swapping proceeds via complete unfolding: A 19F- and 1H- NMR study of the cyanovirin-N protein. J Am Chem Soc 2012; 134: 4229-35

Mishra, Rakesh, Cody L Hoop, Ravindra Kodali, Bankanidhi Sahoo, Patrick C A van der Wel, and Ronald Wetzel. “Serine Phosphorylation Suppresses Huntingtin Amyloid Accumulation by Altering Protein Aggregation Properties.” J Mol Biol 2012; 424: 1–14.

2011

Braunstein MJ, Scott SS, Scott CM, Behrman S, Walter P, Wipf P, Coplan JD, Chrico W, Joseph D, Brodsky JL, Batuman O. Antimyeloma Effects of the Heat Shock Protein 70 Molecular Chaperone Inhibitor MAL3-101. J Oncol. 2011; 2011:232037

X Hu, Z Weng, CT Chu, L Zhang, G Cao, Y Gao, A Signore, J Zhu, T Hastings, JT Greenamyre & J Chen. Peroxiredoxin-2 protects against 6-hydroxydopamine-induced dopaminergic neurodegeneration via attenuation of the ASK1 signaling cascade. J. Neurosci. 2011; 31: 247-261

Needham PG, Mikoluk K, Dhakarwal P, Khadem S, Snyder AC, Subramanya AR, Brodsky JL. The thiazide-sensitive NaCl cotransporter is targeted for chaperone-dependent endoplasmic reticulum-associated degradation. J Biol Chem. 2011;286(51):43611-21

Sivanandam, V.N., Jayaraman, M., Hoop, C.L., Kodali, R., Wetzel, R., & van der Wel, P.C.A., The aggregation-enhancing Huntingtin N-terminus is helical in amyloid fibrils. J. Amer. Chem. Soc. 2011; 133: 4558-4566

Huryn DM, Brodsky JL, Brummond KM, Chambers PG, Eyer B, Ireland AW, Kawasumi M, Laporte MG, Lloyd K, Manteau B, Nghiem P, Quade B, Seguin SP, Wipf P. Chemical methodology as a source of small-molecule checkpoint inhibitors and heat shock protein 70 (Hsp70) modulators. Proc Natl Acad Sci U S A. 2011;108(17):6757-62

VS Van Laar, BA Arnold, SJ Cassady, CT Chu, EA Burton & SB Berman. Bioenergetics of neurons inhibit the translocation response of Parkin following rapid mitochondrial depolarization. Hum Mol Genet 2011; 20: 927-940

Liu L, Gronenborn AM, Bahar I. Longer simulations sample larger subspaces of conformations while maintaining robust mechanisms of motion. Proteins 2011; 80: 616-625

Botha M, Chiang AN, Needham PG, Stephens LL, Hoppe HC, Külzer S, Przyborski JM, Lingelbach K, Wipf P, Brodsky JL, Shonhai A, Blatch GL. Plasmodium falciparum encodes a single cytosolic type I Hsp40 that functionally interacts with Hsp70 and is upregulated by heat shock. Cell Stress Chaperones. 2011;16(4):389-401

Cannon JR, Sew T, Montero L, Burton EA and Greenamyre JT, Pseudotype-dependent lentiviral transduction of astrocytes or neurons in the rat substantia nigra, Exp Neurol. 2011; 228:41-52

Peters KW, Okiyoneda T, Balch WE, Braakman I, Brodsky JL, Guggino WB, Penland CM, Pollard HB, Sorscher EJ, Skach WR, Thomas PJ, Lukacs GL, Frizzell RA. CFTR Folding Consortium: methods available for studies of CFTR folding and correction. Methods Mol Biol. 2011; 742:335-53

Horowitz MP, Milanese C, Di Maio R, Hu X, Montero LM, Sanders LH, Tapias V, Sepe S, van Capellen WA, Burton EA, Greenamyre JT, and Mastroberardino PG. Single-cell redox imaging demonstrates a distinctive response of dopaminergic neurons to oxidative insults, Antioxidants & Redox Signaling 2011; 15:855-71

2010

Wisén S, Bertelsen EB, Thompson AD, Patury S, Ung P, Chang L, Evans CG, Walter GM, Wipf P, Carlson HA, Brodsky JL, Zuiderweg ER, Gestwicki JE. Binding of a small molecule at a protein-protein interface regulates the chaperone activity of hsp70-hsp40. ACS Chem Biol. 2010;5(6):611-22

Buck TM, Kolb AR, Boyd CR, Kleyman TR, Brodsky JL. The endoplasmic reticulum-associated degradation of the epithelial sodium channel requires a unique complement of molecular chaperones. Mol Biol Cell. 2010;21(6):1047-58

2009

Wright CM, Seguin SP, Fewell SW, Zhang H, Ishwad C, Vats A, Lingwood CA, Wipf P, Fanning E, Pipas JM, Brodsky JL. Inhibition of Simian Virus 40 replication by targeting the molecular chaperone function and ATPase activity of T antigen. Virus Res. 2009;141(1):71-80

Thakur, A. K., Jayaraman, M., Mishra, R., Thakur, M., Chellgren, V. M., Byeon, I.-J., Anjum, D. H., Kodali, R., Creamer, T. P., Conway, J. F., Gronenborn, A. M., & Wetzel, R., Polyglutamine disruption of the huntingtin exon1 N-terminus triggers a complex aggregation mechanism. Nature Str. Mol. Biol., 2009; 16: 380-389

Patham B, Duffy J, Lane A, Davis RC, Wipf P, Fewell SW, Brodsky JL, Mensa-Wilmot K. Post-translational import of protein into the endoplasmic reticulum of a trypanosome: an in vitro system for discovery of anti-trypanosomal chemical entities. Biochem J. 2009;419(2):507-17

Liu L, Koharudin LM, Gronenborn AM, Bahar I, A comparative analysis of the equilibrium dynamics of a designed protein inferred from NMR, X-ray, and computations Proteins 2009; 77: 927-939

Chiang AN, Valderramos JC, Balachandran R, Chovatiya RJ, Mead BP, Schneider C, Bell SL, Klein MG, Huryn DM, Chen XS, Day BW, Fidock DA, Wipf P, Brodsky JL. Select pyrimidinones inhibit the propagation of the malarial parasite, Plasmodium falciparum. Bioorg Med Chem. 2009;17(4):1527-33

PG Mastroberardino, EK Hoffman, MP Horowitz, R Betarbet, G Taylor, D Cheng, HM Na, CA Gutekunst, M Gearing, JQ Trojanowski, M Anderson, CT Chu, J Peng, JT Greenamyre. A novel transferrin/TfR2-mediated mitochondrial iron transport system is disrupted in Parkinson's disease. Neurobiol Dis 2009; 34:417-431

H Bayir, A Kapralov, J Jiang, Z Huang, YY Tyurina, VA Tyurin, Q Zhao, NA Belikova, II Vlasova, A Maeda, J Zhu, HM Na, PG Mastroberardino, LJ Sparvero, AA Amoscato, CT Chu, JT Greenamyre & VE Kagan Peroxidase mechanism of lipid dependent cross-linking of synuclein with cytochrome c: Protection against apoptosis versus delayed oxidative stress in Parkinson disease. J Biol Chem 2009; 284: 15951-15969

2008

Rabu C, Wipf P, Brodsky JL, High S. A precursor-specific role for Hsp40/Hsc70 during tail-anchored protein integration at the endoplasmic reticulum. J Biol Chem. 2008;283(41):27504-13

Myerburg MM, McKenna EE, Luke CJ, Frizzell RA, Kleyman TR, Pilewski JM. Prostasin expression is regulated by airway surface liquid volume and is increased in cystic fibrosis. Am J Physiol Lung Cell Mol Physiol. 2008;294(5):L932-41.

Wright CM, Chovatiya RJ, Jameson NE, Turner DM, Zhu G, Werner S, Huryn DM, Pipas JM, Day BW, Wipf P, Brodsky JL. Pyrimidinone-peptoid hybrid molecules with distinct effects on molecular chaperone function and cell proliferation. Bioorg Med Chem. 2008;16(6):3291-301

Bakan, A.; Lazo, J. S.; Wipf, P.; Brummond, K. M.; Bahar, I., Toward a molecular understanding of the interaction of dual specificity phosphatases with substrates: Insights from structure-based modeling and high throughput screening. Curr. Med. Chem. 2008; 15, 2536-2544

2007

Rodina A, Vilenchik M, Moulick K, Aguirre J, Kim J, Chiang A, Litz J, Clement CC, Kang Y, She Y, Wu N, Felts S, Wipf P, Massague J, Jiang X, Brodsky JL, Krystal GW, Chiosis G. Selective compounds define Hsp90 as a major inhibitor of apoptosis in small-cell lung cancer. Nat Chem Biol. 2007;3(8):498-507

Kashlan OB, Mueller GM, Qamar MZ, Poland PA, Ahner A, Rubenstein RC, Hughey RP, Brodsky JL, Kleyman TR. Small heat shock protein alphaA-crystallin regulates epithelial sodium channel expression. J Biol Chem. 2007;282(38):28149-56

Yang L-W, Eyal E, Chennubhotla C, JunGoo J, Gronenborn AM, Bahar I, Insights into Equilibrium Dynamics of Proteins from Comparison of NMR and X-ray Data with Computational Predictions Structure 2007; 15: 741-749

Ahner A, Nakatsukasa K, Zhang H, Frizzell RA, Brodsky JL. Small heat-shock proteins select deltaF508-CFTR for endoplasmic reticulum-associated degradation. Mol Biol Cell. 2007;18(3):806-14

Iyer, AK, Tran KT, Borysenko CW, Cascio M, Camacho CJ, Blair HC, Bahar I, Wells AJ Tenascin cytotactin epidermal growth factor-like repeat binds epidermal growth factor receptor with low affinity J Cell Physiol 2007; 211: 748-58

Bai Q, Garver JA, Hukriede NA, Burton EA. Generation of a transgenic zebrafish model of Tauopathy using a novel promoter element derived from the zebrafish eno2 gene. Nucleic Acids Res. 2007;35(19):6501-16.

2006

Zhang H, Schmidt BZ, Sun F, Condliffe SB, Butterworth MB, Youker RT, Brodsky JL, Aridor M, Frizzell RA. Cysteine string protein monitors late steps in cystic fibrosis transmembrane conductance regulator biogenesis. J Biol Chem. 2006;281(16):11312-21

Wang H, Traub LM, Weixel KM, Hawryluk MJ, Shah N, Edinger RS, Perry CJ, Kester L, Butterworth MB, Peters KW, Kleyman TR, Frizzell RA, Johnson JP. Clathrin-mediated endocytosis of the epithelial sodium channel. Role of epsin. J Biol Chem. 2006 May 19;281(20):14129-35.

Myerburg MM, Butterworth MB, McKenna EE, Peters KW, Frizzell RA, Kleyman TR, Pilewski JM. Airway surface liquid volume regulates ENaC by altering the serine protease-protease inhibitor balance: a mechanism for sodium hyperabsorption in cystic fibrosis. J Biol Chem. 2006;281(38):27942-9.

2004

Fewell SW, Smith CM, Lyon MA, Dumitrescu TP, Wipf P, Day BW, Brodsky JL. Small molecule modulators of endogenous and co-chaperone-stimulated Hsp70 ATPase activity. J Biol Chem. 2004;279(49):51131-40

Brisson, M.; Nguyen, T.; Vogt, A.; Yalowich, J.; Giorgianni, A.; Tobi, D.; Bahar, I.; Stephenson Corey, R. J.; Wipf, P.; Lazo John, S., Discovery and characterization of novel small molecule inhibitors of human cdc25b dual specificity phosphatase. Mol. Pharmacol. 2004; 66, 824-833