Research output per year
Research output per year
- 1 - 50 out of 54 results
Search results
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2024
Disruption of the nascent polypeptide-associated complex leads to reduced polyglutamine aggregation and toxicity
Dublin-Ryan, L. B., Bhadra, A. K. & True, H. L., Aug 2024, In: PloS one. 19, 8 AUGUST, e0303008.Research output: Contribution to journal › Article › peer-review
Open Access -
Genotype‒phenotype correlation in recessive DNAJB4 myopathy
Inoue, M., Jayaraman, D., Bengoechea, R., Bhadra, A., Genetti, C. A., Aldeeri, A. A., Turan, B., Pacheco-Orozco, R. A., Al-Maawali, A., Al Hashmi, N., Zamani, A. G., Göktaş, E., Pekcan, S., Çağlar, H. T., True, H., Beggs, A. H. & Weihl, C. C., Dec 2024, In: Acta Neuropathologica Communications. 12, 1, 171.Research output: Contribution to journal › Article › peer-review
Open Access -
2023
DNAJB6 isoform specific knockdown: Therapeutic potential for limb girdle muscular dystrophy D1
Findlay, A. R., Paing, M. M., Daw, J. A., Haller, M., Bengoechea, R., Pittman, S. K., Li, S., Wang, F., Miller, T. M., True, H. L., Chou, T. F. & Weihl, C. C., Jun 13 2023, In: Molecular Therapy Nucleic Acids. 32, p. 937-948 12 p.Research output: Contribution to journal › Article › peer-review
Open Access3 Scopus citations -
Loss of function variants in DNAJB4 cause a myopathy with early respiratory failure
Weihl, C. C., Töpf, A., Bengoechea, R., Duff, J., Charlton, R., Garcia, S. K., Domínguez-González, C., Alsaman, A., Hernández-Laín, A., Franco, L. V., Sanchez, M. E. P., Beecroft, S. J., Goullee, H., Daw, J., Bhadra, A., True, H., Inoue, M., Findlay, A. R., Laing, N. & Olivé, M. & 2 others, , Jan 2023, In: Acta Neuropathologica. 145, 1, p. 127-143 17 p.Research output: Contribution to journal › Article › peer-review
Open Access13 Scopus citations -
2022
Disease-associated mutations within the yeast DNAJB6 homolog Sis1 slow conformer-specific substrate processing and can be corrected by the modulation of nucleotide exchange factors
Bhadra, A. K., Rau, M. J., Daw, J. A., Fitzpatrick, J. A. J., Weihl, C. C. & True, H. L., Dec 2022, In: Nature communications. 13, 1, 4570.Research output: Contribution to journal › Article › peer-review
Open Access2 Scopus citations -
2020
Client processing is altered by novel myopathy-causing mutations in the HSP40 J domain
Pullen, M. Y., Weihl, C. C. & True, H. L., Jun 2020, In: PloS one. 15, 6, e0234207.Research output: Contribution to journal › Article › peer-review
Open Access7 Scopus citations -
Inhibition of DNAJ-HSP70 interaction improves strength in muscular dystrophy
Bengoechea, R., Findlay, A. R., Bhadra, A. K., Shao, H., Stein, K. C., Pittman, S. K., Daw, J. A. W., Gestwicki, J. E., True, H. L. & Weihl, C. C., Aug 3 2020, In: Journal of Clinical Investigation. 140, 8, p. 4470-4485 16 p.Research output: Contribution to journal › Article › peer-review
Open Access17 Scopus citations -
2019
Lithium chloride corrects weakness and myopathology in a preclinical model of LGMD1D
Findlay, A. R., Bengoechea, R., Pittman, S. K., Chou, T. F., True, H. L. & Weihl, C. C., Apr 1 2019, In: Neurology: Genetics. 5, 2, e318.Research output: Contribution to journal › Article › peer-review
Open Access17 Scopus citations -
2017
Amyloid prions in fungi
Saupe, S. J., Jarosz, D. F. & True, H. L., Sep 5 2017, The Fungal Kingdom. wiley, p. 673-685 13 p.Research output: Chapter in Book/Report/Conference proceeding › Chapter › peer-review
1 Scopus citations -
A toxic imbalance of Hsp70s in Saccharomyces cerevisiae is caused by competition for cofactors
Keefer, K. M. & True, H. L., Sep 2017, In: Molecular Microbiology. 105, 6, p. 860-868 9 p.Research output: Contribution to journal › Article › peer-review
Open Access3 Scopus citations -
Corrigendum: Rapid generation of hypomorphic mutations
Arthur, L. L., Chung, J. J., Janakirama, P., Keefer, K. M., Kolotilin, I., Pavlovic-Djuranovic, S., Chalker, D. L., Grbic, V., Green, R., Menassa, R., True, H. L., Skeath, J. B. & Djuranovic, S., Feb 16 2017, In: Nature communications. 8, p. 14705 1 p., 14705.Research output: Contribution to journal › Article › peer-review
Open Access2 Scopus citations -
Heterologous prion-forming proteins interact to cross-seed aggregation in Saccharomyces cerevisiae
Keefer, K. M., Stein, K. C. & True, H. L., Dec 1 2017, In: Scientific reports. 7, 1, 5853.Research output: Contribution to journal › Article › peer-review
Open Access19 Scopus citations -
Rapid generation of hypomorphic mutations
Arthur, L. L., Chung, J. J., Jankirama, P., Keefer, K. M., Kolotilin, I., Pavlovic-Djuranovic, S., Chalker, D. L., Grbic, V., Green, R., Menassa, R., True, H. L., Skeath, J. B. & Djuranovic, S., Jan 20 2017, In: Nature communications. 8, 14112.Research output: Contribution to journal › Article › peer-review
Open Access13 Scopus citations -
2016
Amyloid prions in fungi
Saupe, S. J., Jarosz, D. F. & True, H. L., 2016, In: Microbiology spectrum. 4, 6, FUNK-0029-2016.Research output: Contribution to journal › Article › peer-review
19 Scopus citations -
Prion-Associated Toxicity is Rescued by Elimination of Cotranslational Chaperones
Keefer, K. M. & True, H. L., Nov 2016, In: PLoS genetics. 12, 11, e1006431.Research output: Contribution to journal › Article › peer-review
Open Access8 Scopus citations -
2015
Myofibrillar disruption and RNA-binding protein aggregation in a mouse model of limb-girdle muscular dystrophy 1D
Bengoechea, R., Pittman, S. K., Tuck, E. P., True, H. L. & Weihl, C. C., Dec 1 2015, In: Human molecular genetics. 24, 23, p. 6588-6602 15 p.Research output: Contribution to journal › Article › peer-review
Open Access30 Scopus citations -
2014
Extensive Diversity of Prion Strains Is Defined by Differential Chaperone Interactions and Distinct Amyloidogenic Regions
Stein, K. C. & True, H. L., May 2014, In: PLoS genetics. 10, 5, e1004337.Research output: Contribution to journal › Article › peer-review
Open Access32 Scopus citations -
Extracellular environment modulates the formation and propagation of particular amyloid structures
Westergard, L. & True, H. L., May 2014, In: Molecular Microbiology. 92, 4, p. 698-715 18 p.Research output: Contribution to journal › Article › peer-review
Open Access18 Scopus citations -
Myopathy-causing mutations in an HSP40 chaperone disrupt processing of specific client conformers
Stein, K. C., Bengoechea, R., Harms, M. B., Weihl, C. C. & True, H. L., Jul 25 2014, In: Journal of Biological Chemistry. 289, 30, p. 21120-21130 11 p.Research output: Contribution to journal › Article › peer-review
Open Access41 Scopus citations -
Prion-like nuclear aggregation of TDP-43 during heat shock is regulated by HSP40/70 chaperones
Udan-Johns, M., Bengoechea, R., Bell, S., Shao, J., Diamond, M. I., True, H. L., Weihl, C. C. & Baloh, R. H., Jan 2014, In: Human molecular genetics. 23, 1, p. 157-170 14 p., ddt408.Research output: Contribution to journal › Article › peer-review
Open Access98 Scopus citations -
Prion Strains and Amyloid Polymorphism Influence Phenotypic Variation
Stein, K. C. & True, H. L., Sep 1 2014, In: PLoS pathogens. 10, 9Research output: Contribution to journal › Article › peer-review
Open Access23 Scopus citations -
Regulation of the Hsp104 middle domain activity is critical for yeast prion propagation
Dulle, J. E., Stein, K. C. & True, H. L., Jan 23 2014, In: PloS one. 9, 1, e87521.Research output: Contribution to journal › Article › peer-review
Open Access15 Scopus citations -
Structural variants of yeast prions show conformer-specific requirements for chaperone activity
Stein, K. C. & True, H. L., Sep 1 2014, In: Molecular Microbiology. 93, 6, p. 1156-1171 16 p.Research output: Contribution to journal › Article › peer-review
Open Access24 Scopus citations -
Wild yeast harbour a variety of distinct amyloid structures with strong prion-inducing capabilities
Westergard, L. & True, H. L., Apr 2014, In: Molecular Microbiology. 92, 1, p. 183-193 11 p.Research output: Contribution to journal › Article › peer-review
Open Access19 Scopus citations -
2013
Low activity of select Hsp104 mutants is sufficient to propagate unstable prion variants
Dulle, J. E. & True, H. L., Sep 2013, In: Prion. 7, 5, p. 394-403 10 p.Research output: Contribution to journal › Article › peer-review
Open Access6 Scopus citations -
Soluble oligomers are sufficient for transmission of a yeast prion but do not confer phenotype
Dulle, J. E., Bouttenot, R. E., Underwood, L. A. & True, H. L., Oct 28 2013, In: Journal of Cell Biology. 203, 2, p. 197-204 8 p.Research output: Contribution to journal › Article › peer-review
Open Access14 Scopus citations -
Spontaneous Variants of the [RNQ+] Prion in Yeast Demonstrate the Extensive Conformational Diversity Possible with Prion Proteins
Huang, V. J., Stein, K. C. & True, H. L., Oct 25 2013, In: PloS one. 8, 10, e79582.Research output: Contribution to journal › Article › peer-review
Open Access28 Scopus citations -
2011
The [RNQ+] prion: A model of both functional and pathological amyloid
Stein, K. C. & True, H. L., Oct 2011, In: Prion. 5, 4, p. 291-298 8 p.Research output: Contribution to journal › Review article › peer-review
24 Scopus citations -
2010
Analysis of the [RNQ+] prion reveals stability of amyloid fibers as the key determinant of yeast prion variant propagation
Kalastavadi, T. & True, H. L., Jul 2 2010, In: Journal of Biological Chemistry. 285, 27, p. 20748-20755 8 p.Research output: Contribution to journal › Article › peer-review
Open Access21 Scopus citations -
The spontaneous appearance rate of the yeast prion [PSI+] and its implications for the evolution of the evolvability properties of the [PSI+] system
Lancaster, A. K., Bardill, J. P., True, H. L. & Masel, J., Feb 2010, In: Genetics. 184, 2, p. 393-400 8 p.Research output: Contribution to journal › Article › peer-review
Open Access90 Scopus citations -
The Sua5 protein is essential for normal translational regulation in yeast
Lin, C. A., Ellis, S. R. & True, H. L., Jan 2010, In: Molecular and cellular biology. 30, 1, p. 354-363 10 p.Research output: Contribution to journal › Article › peer-review
65 Scopus citations -
2009
Disease-associated mutant ubiquitin causes proteasomal impairment and enhances the toxicity of protein aggregates
Tank, E. M. H. & True, H. L., Feb 2009, In: PLoS genetics. 5, 2, e1000382.Research output: Contribution to journal › Article › peer-review
Open Access47 Scopus citations -
Heterologous Prion Interactions Are Altered by Mutations in the Prion Protein Rnq1p
Bardill, J. P. & True, H. L., May 8 2009, In: Journal of Molecular Biology. 388, 3, p. 583-596 14 p.Research output: Contribution to journal › Article › peer-review
Open Access23 Scopus citations -
Mutants of the pafl complex alter phenotypic expression of the yeast prion [PSI+]
Strawn, L. A., Lin, C. A., Tank, E. M. H., Osman, M. M., Simpson, S. A. & True, H. L., Apr 15 2009, In: Molecular biology of the cell. 20, 8, p. 2229-2241 13 p.Research output: Contribution to journal › Article › peer-review
Open Access5 Scopus citations -
Requirements of Hsp104p activity and Sis1p binding for propagation of the [RNQ+] prion
Bardill, J. P., Dulle, J. E., Fisher, J. R. & True, H. L., 2009, In: Prion. 3, 3, p. 151-160 10 p.Research output: Contribution to journal › Article › peer-review
Open Access15 Scopus citations -
2008
Insights into intragenic and extragenic effectors of prion propagation using chimeric prion proteins.
True, H. L., Kalastavadi, T. & Tank, E. M. H., Apr 2008, In: Prion. 2, 2, p. 45-47 3 p.Research output: Contribution to journal › Review article › peer-review
Open Access -
Prion protein insertional mutations increase aggregation propensity but not fiber stability
Kalastavadi, T. & True, H. L., 2008, In: BMC Biochemistry. 9, 1, 7.Research output: Contribution to journal › Article › peer-review
Open Access9 Scopus citations -
2007
Prion protein repeat expansion results in increased aggregation and reveals phenotypic variability
Tank, E. M. H., Harris, D. A., Desai, A. A. & True, H. L., Aug 2007, In: Molecular and cellular biology. 27, 15, p. 5445-5455 11 p.Research output: Contribution to journal › Article › peer-review
Open Access39 Scopus citations -
2006
Deletion of RNQ1 gene reveals novel functional relationship between divergently transcribed Bik1p/CLIP-170 and Sfi1p in spindle pole body separation
Strawn, L. A. & True, H. L., Dec 2006, In: Current Genetics. 50, 6, p. 347-366 20 p.Research output: Contribution to journal › Review article › peer-review
10 Scopus citations -
New Insights into Prion Structure and Toxicity
Harris, D. A. & True, H. L., May 4 2006, In: Neuron. 50, 3, p. 353-357 5 p.Research output: Contribution to journal › Short survey › peer-review
Open Access104 Scopus citations -
The battle of the fold: Chaperones take on prions
True, H. L., Feb 2006, In: Trends in Genetics. 22, 2, p. 110-117 8 p.Research output: Contribution to journal › Review article › peer-review
40 Scopus citations -
2004
Epigenetic regulation of translation reveals hidden genetic variation to produce complex traits
True, H. L., Bedin, I. & Lindquist, S. L., Sep 9 2004, In: Nature. 431, 7005, p. 184-187 4 p.Research output: Contribution to journal › Article › peer-review
291 Scopus citations -
2001
Molecular population genetics and evolution of a prion-like protein in saccharomyces cerevisiae
Jensen, M. A., True, H. L., Chernoff, Y. O. & Lindquist, S., 2001, In: Genetics. 159, 2, p. 527-535 9 p.Research output: Contribution to journal › Article › peer-review
50 Scopus citations -
Novel RNA-binding Properties of Pop3p Support a Role for Eukaryotic RNase P Protein Subunits in Substrate Recognition
Brusca, E. M., True, H. L. & Celander, D. W., Nov 9 2001, In: Journal of Biological Chemistry. 276, 45, p. 42543-42548 6 p.Research output: Contribution to journal › Article › peer-review
Open Access10 Scopus citations -
Remodeling of yeast genome expression in response to environmental changes
Causton, H. C., Ren, B., Sang Seok Koh, S. K., Harbison, C. T., Kanin, E., Jennings, E. G., Tong Ihn Lee, I. L., True, H. L., Lander, E. S. & Young, R. A., 2001, In: Molecular biology of the cell. 12, 2, p. 323-337 15 p.Research output: Contribution to journal › Article › peer-review
Open Access1116 Scopus citations -
2000
[22] RNA challenge phages as genetic tools for study of RNA-ligand interactions
Celander, D. W., Bennett, K. A., Fouts, D. E., Seitz, E. A. & True, H. L., 2000, In: Methods in enzymology. 318, p. 332-350 19 p.Research output: Contribution to journal › Review article › peer-review
4 Scopus citations -
A yeast prion provides a mechanism for genetic variation and phenotypic diversity
True, H. L. & Lindquist, S. L., Sep 28 2000, In: Nature. 407, 6803, p. 477-483 7 p.Research output: Contribution to journal › Article › peer-review
573 Scopus citations -
1999
Essential components of the Ti plasmid trb system, a type IV macromolecular transporter
Li, P. L., Hwang, I., Miyagi, H., True, H. & Farrand, S. K., Aug 1999, In: Journal of bacteriology. 181, 16, p. 5033-5041 9 p.Research output: Contribution to journal › Article › peer-review
Open Access49 Scopus citations -
1998
Protein components contribute to active site architecture for eukaryotic ribonuclease P
True, H. L. & Celander, D. W., Mar 27 1998, In: Journal of Biological Chemistry. 273, 13, p. 7193-7196 4 p.Research output: Contribution to journal › Article › peer-review
Open Access16 Scopus citations -
1997
Direct genetic selection of two classes of R17/MS2 coat proteins with altered capsid assembly properties and expanded RNA-binding activities
Wang, S., True, H. L., Seitz, E. M., Bennett, K. A., Fouts, D. E., Gardner, J. F. & Celander, D. W., Apr 15 1997, In: Nucleic acids research. 25, 8, p. 1649-1657 9 p.Research output: Contribution to journal › Article › peer-review
Open Access11 Scopus citations