Bragg, R. M., Mathews, E. W., Grindeland, A., Cantle, J. P., Howland, D., Vogt, T., & Carroll, J. B. (2024). Global huntingtin knockout in adult mice leads to fatal neurodegeneration that spares the pancreas. Life Science Alliance, 7(9).
Bragg, R. M., Coffey, S. R., Cantle, J. P., Hu, S., Singh, S., Legg, S. R., … & Carroll, J. B. (2023). Huntingtin loss in hepatocytes is associated with altered metabolism, adhesion, and liver zonation. Life Science Alliance, 6(11).
Gleeson, J. G., Bennett, C. F., Carroll, J. B., Cole, T., Douville, J., Glass, S., … & Crooke, S. T. (2023). Personalized antisense oligonucleotides ‘for free, for life’—the n-Lorem Foundation. Nature Medicine, 1-2.
Zhao X, McHugh C, Coffey S, Jimenez DA, Carroll JB, Usdin K. Expansion in stool is a sensitive, non-invasive marker of somatic expansion in multiple mouse models of the repeat expansion diseases. Dis Model Mech. 15(5):dmm049453.
Greco TM, Secker C, Ramos ES, Federspiel JD, Liu JP, Perez AM, Al-Ramahi I, Cantle JP, Carroll JB, Botas J, Zeitlin SO, Wanker EE, Cristea IM. Dynamics of huntingtin protein interactions in the striatum identifies candidate modifiers of Huntington disease. Cell Syst; 20;13(4):304-320.e5.
Harding RJ, Deme J, Hevler JH, Tamara S, Lemak A, Cantle JP, Szewczyk M, Zuo X, Fan L, Schapira M, Carroll JB, Heck AJR, Lea SM, Arrowsmith CH. HAP40 orchestrates huntingtin 3D structure for differential interaction with polyglutamine expanded exon 1. Commun Biology. 4(1):1374.
Coffey SR, Andrew M, Ging H, Hamilton J, Flower M, Kovalenko M, Bragg RM, Cantle JP, Carrillo JP, McHugh CA, Rodier JA, Marchionini DM, Wilkinson HA, Kwak S, Howland DS, Bennett CF, Mouro Pinto R, Auburger G, Zeitlin SO, Kordasiewicz HB, Tabrizi SJ, Wheeler VC, Carroll JB. Huntingtin lowering reduces somatic instability at CAG-expanded loci. https://www.biorxiv.org/content/10.1101/2020.07.23.218347v1.
Pearl JR, Shetty AC, Cantle JP, Bergey DE, Toor A, Bragg RM, Coffey SC, Kordasiewicz HB, Hood LE, Price ND, Ament SA, Carroll JB. Altered Huntingtin-Chromatin Interactions Predict Transcriptional and Epigenetic Changes in Huntington’s Disease. https://doi.org/10.1101/2020.06.04.132571.
Minikel EV, Zhao HT, Le J, O’Moore J, Pitstick R, Graffam S, Carlson GA, Kriz J, Kim JB, Ma J, Wille H, Aiken J, McKenzie D, Doh-ura K, Beck M, O’Keefe R, Stathopoulos J, Carson T, Schreiber SL, Carroll JB, Kordasiewicz HB, Cabin DE, Vallabh SM. Prion protein lowering is a disease-modifying therapy across prion strains, disease stages, and endpoints. Nucleic Acids Res. 2020;48(19):gkaa616-.
Malaiya S, Cortes-Gutierrez M, Herb BR, Coffey SR, Legg SRW, Cantle JP, Colantuoni C., Carroll JB, Ament, SA. Single-Nucleus RNA-Seq Reveals Dysregulation of Striatal Cell Identity Due to Huntington’s Disease Mutations. J Neurosci. 2021;41(25):5534–52.
Raymond GJ, Zhao HT, Race B, Raymond LD, Williams K, Swayze E, Graffam S, Le J, Caron T, Stathopoulos J, O’Keefe R, Lubke LL, Schreiber SL, Mazur C, Cabin DE, Carroll JB, Minikel EV, Holly Kordasiewicz H, Caughey, Vallabh SM, Antisense oligonucleotides extend survival of prion-infected mice. JCI Insight. pii: 131175. doi: 10.1172/jci.insight.131175
Minnig S, Bragg RM, Tiwana HS, Solem WT, Hovander WS, Vik E, Hamilton M,Coffey SC, Cantle JP, Carroll JB. Early Detection of Apathetic Phenotypes in Huntington’s Disease Knock-in Mice Using Open Source Tools. Scientific Reports. 8(1):2304.
Ament SA, Pearl JR, Cantle JP, Bragg RM, Skene PJ, Coffey SR, Bergey DE, Wheeler VC, MacDonald ME, Baliga NS, Rosinski J, Hood LE, Carroll JB, Price ND. Transcriptional regulatory networks underlying gene expression changes in Huntington’s disease. Mol Syst Biol. 14(3):e7435.
Kovalenko M, Milnerwood A, Giordano J, St Claire J, Guide JR, Stromberg M, Gillis T, Sapp E, DiFiglia M, MacDonald ME, Carroll JB, Lee JM, Tappan S, Raymond L, Wheeler VC. HttQ111/+ Huntington’s Disease Knock-in Mice Exhibit Brain Region-Specific Morphological Changes and Synaptic Dysfunction. Journal of Huntington’s disease. 7(1):17-33.
Coffey SR, Bragg RM, Minnig S, Ament SA, Cantle JP, Glickenhaus A, Shelnut D, Carrillo JM, Shuttleworth DD, Rodier JA, Noguchi K, Bennett CF, Price ND, Kordasiewicz HB, Carroll JB. Peripheral Htt silencing does not ameliorate central signs of disease in the B6.HttQ111/+ mouse model of Huntington’s Disease. PLOS ONE 2017 Apr 28;12(4):e0175968.
Bragg RM, Coffey SR, Weston RM, Ament SA, Cantle JP, Minnig S, Funk CC, Shuttleworth DD, Woods EL, Sullivan BR, Jones L. Glickenhaus A, Anderson JS, Anderson MD, Dunnett SB, Wheeler VC, MacDonald ME, Brooks SP, Price ND, Carroll JB. Motivational, proteostatic and transcriptional deficits precede synapse loss, gliosis and neurodegeneration in the B6. HttQ111/+ model of Huntington’s disease. Scientific Reports. 7:41570.
Ament SA, Pearl JP, Grindeland A, St Clair J, Earls JC, Kovalenko M, Gillis T, Mysore J, Gusella JF, Lee JM, Kwak S, Howland D, Lee MY, Baxter D, Scherler K, Wang K, German D, Carroll JB, MacDonald ME, Carlson G, Wheeler VC, Price ND, Hood LE. High resolution time-course mapping of early transcriptomic, molecular and cellular phenotypes in Huntington’s disease CAG knock-in mice across multiple genetic backgrounds. Hum Mol Gen. 26(5):913-22.
Carroll JB, Bates GP, Steffan J, Saft C, Tabrizi SJ. Treating the whole body in Huntington’s disease. The Lancet Neurology. 14(11):1135-42.
Carroll JB, Deik A, Fossale E, Weston RM, Guide JR, Arjomand J, Kwak S, Clish CB, MacDonald ME. HdhQ111 Mice Exhibit Tissue Specific Metabolite Profiles that Include Striatal Lipid Accumulation. PLOS ONE. doi: 10(8):e0134465.
Southwell AL, Skotte NH, Kordasiewicz HB, Østergaard ME, Watt AT, Carroll JB … Hayden MR. In vivo evaluation of candidate allele-specific mutant huntingtin gene silencing antisense oligonucleotides. Molecular Therapy. 22(12):2093-106.
Skotte NH, Southwell AL, Østergaard ME, Carroll JB, Warby SC, Doty CN, Petoukhov E, Vaid K, Kordasiewicz H, Watt AT, Freier SM, Hung G, Seth PP, Bennett CF, Swayze EE, Hayden MR. Allele-specific suppression of mutant huntingtin using antisense oligonucleotides: providing a therapeutic option for all Huntington disease patients. PLOS ONE. 9(9):e107434.
Wild EJ, Carroll JB. HDBuzz: empowering patients through accessible education. Trends in Molecular Medicine. 18(1):1-3.
Southwell AL, Warby SC, Carroll JB, Doty CN, Skotte NH, Zhang W, Villanueva EB, Kovalik V, Xie Y, Pouladi MA, Collins JA, Yang XW, Franciosi S, Hayden MR. A fully humanized transgenic mouse model of Huntington disease. Hum Mol Genet. 22(1):18-34.
Carroll JB, Warby SC, Southwell AL, Doty CN, Greenlee S, Skotte N, Hung G, Bennett CF, Freier SM, Hayden MR. Potent and selective antisense oligonucleotides targeting single-nucleotide polymorphisms in the Huntington disease gene. Molecular Therapy. 19(12):2178-85.
Carroll JB, Southwell AL, Graham RK, Lerch JP, Ehrnhoefer DE, Cao LP, Zhang WN, Deng Y, Bissada N, Henkelman RM, Hayden MR. Mice lacking caspase-2 are protected from behavioral changes, but not pathology, in the YAC128 model of Huntington disease. Molecular Neurodegeneration. 6(1):59.
Carroll JB, Lerch JP, Franciosi S, Spreeuw A, Bissada N, Henkelman RM, Hayden MR. Natural history of disease in the YAC128 mouse reveals a discrete signature of pathology in Huntington disease. Neurobiology of Disease. 43(1):257-65.
Huang K, Sanders SS, Kang R, Carroll JB Sutton L, Wan J, Singaraja R, Young FB, Liu L, El-Husseini A, Davis NG, Hayden MR. Wildtype HTT modulates the enzymatic activity of the neuronal palmitoyl transferase HIP14. Hum Mol Genet. 20(17):3356-65.
Graham RK, Deng Y, Carroll J, Vaid K, Cowan C, Poualdi MA, Metzler M, Bissada N, Wang L, Faul RL, Gray M, Yang XM, Raymond LA, Hayden MR. Cleavage at the 586 amino acid caspase-6 site in mutant huntingtin influences caspase-6 activation in vivo. J Neurosci. 30(45):15019-29.
Valenza M, Leoni V, Karasinska JM, Petricca L, Fan J, Carroll J, Pouladi MA, Fossale E, Nguyen HP, Riess O, MacDonald ME, Wellington C, DiDonato S, Hayden MR, Cattaneo E. Cholesterol defect is marked across multiple rodent models of Huntington’s disease and is manifest in astrocytes. J Neurosci. 30(32):10844-50.
Joshi PR, Wu NP, André VM, Cummings DM, Cepeda C, Joyce JA, Carroll JB, Leavitt BR, Hayden MR, Levine MS, Bamford NS. Age-dependent alterations of corticostriatal activity in the YAC128 mouse model of Huntington disease. J Neurosci. 29(8):2414-27.
Lerch JP, Carroll JB, Dorr A, Spring S, Evans AC, Hayden MR, Sled JG, Henkelman RM. Cortical thickness measured from MRI in the YAC128 mouse model of Huntington’s disease. Neuroimage. 41(2):243-51.
Warby SC, Doty CN, Graham RK, Carroll JB, Yang YZ, Singaraja RR, Overall CM, Hayden MR. Activated caspase-6 and caspase-6-cleaved fragments of huntingtin specifically colocalize in the nucleus. Human Molecular Genetics. 17(15):2390-404.
Valenza M, Carroll JB*, Leoni V, Bertram LN, Björkhem I, Singaraja RR, Di Donato S, Lutjohann D, Hayden MR, Cattaneo E. Cholesterol biosynthesis pathway is disturbed in YAC128 mice and is modulated by huntingtin mutation. Human Molecular Genetics. 16(18):2187-98. * Equal Contribution
Lerch JP, Carroll JB*, Spring S, Bertram LN, Schwab C, Hayden MR, Henkelman RM. Automated deformation analysis in the YAC128 Huntington disease mouse model. Neuroimage. 16(18):2187-98. * Equal Contribution
Van Raamsdonk JM, Metzler M, Slow E, Pearson J, Schwab C, Carroll J, Graham RK, Leavitt BR, Hayden MR. Phenotypic abnormalities in the YAC128 mouse model of Huntington disease are penetrant on multiple genetic backgrounds and modulated by strain. Neurobiology of Disease. 26(1):189-200.
Cohen S, Behzad AR, Carroll JB, Pante N. Parvoviral nuclear import: bypassing the host nuclear-transport machinery. Journal of General Virology. 87(11):3209-13.