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Research Output 1989 2019

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2019

Distinct mechanical properties in homologous spectrin-like repeats of utrophin

Rajaganapathy, S., McCourt, J. L., Ghosal, S., Lindsay, A., McCourt, P. M., Lowe, D. A., Ervasti, J. M. & Salapaka, M. V., Dec 1 2019, In : Scientific reports. 9, 1, 5210.

Research output: Contribution to journalArticle

Open Access
Utrophin
Spectrin
Dystrophin
Dystroglycans
Connectin
1 Citation (Scopus)

Dystrophinopathy-associated dysfunction of Krebs cycle metabolism

Lindsay, A., Chamberlain, C. M., Witthuhn, B. A., Lowe, D. A. & Ervasti, J. M., Jan 1 2019, In : Human molecular genetics. 28, 6, p. 942-951 10 p.

Research output: Contribution to journalArticle

Citric Acid Cycle
Succinic Acid
Dystrophin
Muscles
Inbred mdx Mouse
1 Citation (Scopus)

Isometric resistance training increases strength and alters histopathology of dystrophin-deficient mouse skeletal muscle

Lindsay, A., Larson, A. A., Verma, M., Ervasti, J. M. & Lowe, D. A., Feb 1 2019, In : Journal of applied physiology. 126, 2, p. 363-375 13 p.

Research output: Contribution to journalArticle

Open Access
Dystrophin
Resistance Training
Duchenne Muscular Dystrophy
Skeletal Muscle
Muscles
3 Citations (Scopus)

Persistent upregulation of the β-tubulin tubb6, linked to muscle regeneration, is a source of microtubule disorganization in dystrophic muscle

Randazzo, D., Khalique, U., Belanto, J. J., Kenea, A., Talsness, D. M., Olthoff, J. T., Tran, M. D., Zaal, K. J., Pak, K., Pinal-Fernandez, I., Mammen, A. L., Sackett, D., Ervasti, J. M. & Ralston, E., Jan 1 2019, In : Human molecular genetics. 28, 7, p. 1117-1135 19 p.

Research output: Contribution to journalArticle

Open Access
Tubulin
Microtubules
Regeneration
Up-Regulation
Muscles

Variable cytoplasmic actin expression impacts the sensitivity of different dystrophin-deficient mdx skeletal muscles to eccentric contraction

Lindsay, A., Southern, W. M., McCourt, P. M., Larson, A. A., Hodges, J. S., Lowe, D. A. & Ervasti, J. M., Jul 1 2019, In : FEBS Journal. 286, 13, p. 2562-2576 15 p.

Research output: Contribution to journalArticle

Utrophin
Dystrophin
Muscle
Actins
Skeletal Muscle
2018
5 Citations (Scopus)

Dystrophin As a Molecular Shock Absorber

Le, S., Yu, M., Hovan, L., Zhao, Z., Ervasti, J. M. & Yan, J., Dec 26 2018, In : ACS nano. 12, 12, p. 12140-12148 9 p.

Research output: Contribution to journalArticle

shock absorbers
Shock absorbers
Dystrophin
Muscle
muscular function
3 Citations (Scopus)

Essential nucleotide- and protein-dependent functions of Actb/β-actin

Patrinostro, X., Roy, P., Lindsay, A., Chamberlain, C. M., Sundby, L. J., Starker, C., Voytas, D. F., Ervasti, J. M. & Perrin, B. J., Jul 31 2018, In : Proceedings of the National Academy of Sciences of the United States of America. 115, 31, p. 7973-7978 6 p.

Research output: Contribution to journalArticle

Actins
Nucleotides
Stereocilia
Proteins
High-Frequency Hearing Loss
5 Citations (Scopus)

Impaired muscle relaxation and mitochondrial fission associated with genetic ablation of cytoplasmic actin isoforms

O'Rourke, A. R., Lindsay, A., Tarpey, M. D., Yuen, S., McCourt, P., Nelson, D. M., Perrin, B. J., Thomas, D. D., Spangenburg, E. E., Lowe, D. A. & Ervasti, J. M., Feb 2018, In : FEBS Journal. 285, 3, p. 481-500 20 p.

Research output: Contribution to journalArticle

Open Access
Mitochondrial Dynamics
Muscle Relaxation
Ablation
Muscle
Actins

Integrative effects of dystrophin loss on metabolic function of the mdx mouse

Strakova, J., Kamdar, F., Kulhanek, D., Razzoli, M., Garry, D. J., Ervasti, J. M., Bartolomucci, A. & Townsend, D. W., Dec 1 2018, In : Scientific reports. 8, 1, 13624.

Research output: Contribution to journalArticle

Inbred mdx Mouse
Dystrophin
Duchenne Muscular Dystrophy
Skeletal Muscle
Fats
4 Citations (Scopus)

Loss of peroxiredoxin-2 exacerbates eccentric contraction-induced force loss in dystrophin-deficient muscle

Olthoff, J. T., Lindsay, A., Abo-Zahrah, R., Baltgalvis, K. A., Patrinostro, X., Belanto, J. J., Yu, D. Y., Perrin, B. J., Garry, D. J., Rodney, G. G., Lowe, D. A. & Ervasti, J. M., Dec 1 2018, In : Nature communications. 9, 1, 5104.

Research output: Contribution to journalArticle

Open Access
Peroxiredoxins
Dystrophin
eccentrics
muscles
contraction
5 Citations (Scopus)

Mouse models of two missense mutations in actin-binding domain 1 of dystrophin associated with duchenne or becker muscular dystrophy

McCourt, J. L., Talsness, D. M., Lindsay, A., Arpke, R. W., Chatterton, P. D., Nelson, D. M., Chamberlain, C. M., Olthoff, J. T., Belanto, J. J., McCourt, P. M., Kyba, M., Lowe, D. A. & Ervasti, J. M., Feb 1 2018, In : Human molecular genetics. 27, 3, p. 451-462 12 p.

Research output: Contribution to journalArticle

Open Access
Dystrophin
Duchenne Muscular Dystrophy
Missense Mutation
Actins
Proteasome Endopeptidase Complex
8 Citations (Scopus)

Neopterin/7,8-dihydroneopterin is elevated in Duchenne muscular dystrophy patients and protects mdx skeletal muscle function

Lindsay, A., Schmiechen, A., Chamberlain, C. M., Ervasti, J. M. & Lowe, D. A., Jul 1 2018, In : Experimental Physiology. 103, 7, p. 995-1009 15 p.

Research output: Contribution to journalArticle

Open Access
Neopterin
Duchenne Muscular Dystrophy
Skeletal Muscle
Creatinine
Muscles
12 Citations (Scopus)

Variable rescue of microtubule and physiological phenotypes in mdx muscle expressing different miniaturized dystrophins

Nelson, D. M., Lindsay, A., Judge, L. M., Duan, D., Chamberlain, J. S., Lowe, D. A. & Ervasti, J. M., Jun 15 2018, In : Human molecular genetics. 27, 12, p. 2090-2100 11 p.

Research output: Contribution to journalArticle

Open Access
Dystrophin
Microtubules
Phenotype
Muscles
Inbred mdx Mouse
4 Citations (Scopus)

Xanthine oxidase is hyper-active in Duchenne muscular dystrophy

Lindsay, A., McCourt, P. M., Karachunski, P. I., Lowe, D. A. & Ervasti, J. M., Dec 1 2018, In : Free Radical Biology and Medicine. 129, p. 364-371 8 p.

Research output: Contribution to journalArticle

Duchenne Muscular Dystrophy
Xanthine Oxidase
Inbred mdx Mouse
Dystrophin
Muscle
2017
9 Citations (Scopus)

Relative importance of βcyto-and γcyto-actin in primary mouse embryonic fibroblasts

Patrinostro, X., O'Rourke, A. R., Chamberlain, C. M., Moriarity, B. S., Perrin, B. J. & Ervasti, J. M., Mar 15 2017, In : Molecular biology of the cell. 28, 6, p. 771-782 12 p.

Research output: Contribution to journalArticle

Actins
Fibroblasts
Gene Targeting
Protein Isoforms
Serum Response Factor
2016
24 Citations (Scopus)

Actin Is Crucial for All Kinetically Distinguishable Forms of Endocytosis at Synapses

Wu, X. S., Lee, S. H., Sheng, J., Zhang, Z., Zhao, W. D., Wang, D., Jin, Y., Charnay, P., Ervasti, J. M. & Wu, L. G., Dec 7 2016, In : Neuron. 92, 5, p. 1020-1035 16 p.

Research output: Contribution to journalArticle

Endocytosis
Synapses
Actins
Electron Microscopy
Protein Isoforms
6 Citations (Scopus)

High frequency hearing loss and hyperactivity in DUX4 transgenic mice

Dandapat, A., Perrin, B. J., Cabelka, C., Razzoli, M., Ervasti, J. M., Bartolomucci, A., Lowe, D. A. & Kyba, M., Mar 1 2016, In : PloS one. 11, 3, e0151467.

Research output: Contribution to journalArticle

High-Frequency Hearing Loss
Audition
hearing
Transgenic Mice
Doxycycline
17 Citations (Scopus)

Independent variability of microtubule perturbations associated with dystrophinopathy

Belanto, J. J., Olthoff, J. T., Mader, T. L., Chamberlain, C. M., Nelson, D. M., McCourt, P. M., Talsness, D. M., Gundersen, G. G., Lowe, D. A. & Ervasti, J. M., Nov 15 2016, In : Human molecular genetics. 25, 22, p. 4951-4961 11 p.

Research output: Contribution to journalArticle

Dystrophin
Microtubules
Inbred mdx Mouse
Utrophin
Tubulin
2015
5 Citations (Scopus)

Creatine kinase B is necessary to limit myoblast fusion during myogenesis

Simionescu-Bankston, A., Pichavant, C., Canner, J. P., Apponi, L. H., Wang, Y., Steeds, C., Olthoff, J. T., Belanto, J. J., Ervasti, J. M. & Pavlath, G. K., Jun 1 2015, In : American Journal of Physiology - Cell Physiology. 308, 11, p. C919-C931

Research output: Contribution to journalArticle

Muscle Development
Myoblasts
Creatine Kinase
Skeletal Muscle Fibers
Actins
4 Citations (Scopus)

Disease-proportional proteasomal degradation of missense dystrophins

Talsness, D. M., Belanto, J. J. & Ervasti, J. M., Oct 6 2015, In : Proceedings of the National Academy of Sciences of the United States of America. 112, 40, p. 12414-12419 6 p.

Research output: Contribution to journalArticle

Dystrophin
Mutation
Frameshift Mutation
Missense Mutation
Phenotype

In vitro stability of therapeutically relevant, internally truncated dystrophins

McCourt, J. L., Rhett, K. K., Jaeger, M. A., Belanto, J. J., Talsness, D. M. & Ervasti, J. M., Apr 28 2015, (Accepted/In press) In : Skeletal Muscle. p. 1-10 10 p.

Research output: Contribution to journalArticle

Dystrophin
Exons
Genetic Therapy
Duchenne Muscular Dystrophy
Circular Dichroism
5 Citations (Scopus)

In vitro stability of therapeutically relevant, internally truncated dystrophins

McCourt, J. L., Rhett, K. K., Jaeger, M. A., Belanto, J. J., Talsness, D. M. & Ervasti, J. M., Apr 28 2015, In : Skeletal Muscle. 5, 1, 13.

Research output: Contribution to journalArticle

Dystrophin
Exons
Genetic Therapy
Duchenne Muscular Dystrophy
Circular Dichroism
28 Citations (Scopus)

Length regulation of mechanosensitive stereocilia depends on very slow actin dynamics and filament-severing proteins

Narayanan, P., Chatterton, P., Ikeda, A., Ikeda, S., Corey, D. P., Ervasti, J. M. & Perrin, B. J., Apr 21 2015, In : Nature communications. 6, 6855.

Research output: Contribution to journalArticle

Stereocilia
Actin Cytoskeleton
Actins
filaments
hair
1 Citation (Scopus)

Postanesthetic Death in a Cat With Myopathy

Remmers, G., Hayden, D. W., Jaeger, M. A., Ervasti, J. M. & Valberg, S. J., Jan 29 2015, In : Veterinary pathology. 52, 1, p. 186-188 3 p.

Research output: Contribution to journalArticle

muscular dystrophy
Muscular Dystrophies
muscular diseases
Muscular Diseases
dystrophin
2014
55 Citations (Scopus)

Microtubule binding distinguishes dystrophin from utrophin

Belanto, J. J., Mader, T. L., Eckhoff, M. D., Strandjord, D. M., Banks, G. B., Gardner, M. K., Lowe, D. A. & Ervasti, J. M., Apr 15 2014, In : Proceedings of the National Academy of Sciences of the United States of America. 111, 15, p. 5723-5728 6 p.

Research output: Contribution to journalArticle

Utrophin
Dystrophin
Microtubules
Inbred mdx Mouse
Duchenne Muscular Dystrophy
11 Citations (Scopus)

The ZZ domain of dystrophin in DMD: Making sense of missense mutations

Vulin, A., Wein, N., Strandjord, D. M., Johnson, E. K., Findlay, A. R., Maiti, B., Howard, M. T., Kaminoh, Y. J., Taylor, L. E., Simmons, T. R., Ray, W. C., Montanaro, F., Ervasti, J. M. & Flanigan, K. M., Feb 1 2014, In : Human mutation. 35, 2, p. 257-264 8 p.

Research output: Contribution to journalArticle

Dystroglycans
Dystrophin
Duchenne Muscular Dystrophy
Missense Mutation
Mutation
2013
87 Citations (Scopus)

An ex vivo gene therapy approach to treat muscular dystrophy using inducible pluripotent stem cells

Filareto, A., Parker, S., Darabi, R., Borges, L., Iacovino, M., Schaaf, T., Mayerhofer, T., Chamberlain, J. S., Ervasti, J. M., McIvor, R. S., Kyba, M. & Perlingeiro, R. C. R., Apr 11 2013, In : Nature communications. 4, 1549.

Research output: Contribution to journalArticle

Utrophin
gene therapy
Gene therapy
Induced Pluripotent Stem Cells
Pluripotent Stem Cells
22 Citations (Scopus)

Identification of New Dystroglycan Complexes in Skeletal Muscle

Johnson, E. K., Li, B., Yoon, J. H., Flanigan, K. M., Martin, P. T., Ervasti, J. M. & Montanaro, F., Aug 8 2013, In : PloS one. 8, 8, e73224.

Research output: Contribution to journalArticle

Dystroglycans
dystrophin
Dystrophin
Muscle
skeletal muscle
36 Citations (Scopus)

β-actin and fascin-2 cooperate to maintain stereocilia length

Perrin, B. J., Strandjord, D. M., Narayanan, P., Henderson, D. M., Johnson, K. R. & Ervasti, J. M., May 13 2013, In : Journal of Neuroscience. 33, 19, p. 8114-8121 8 p.

Research output: Contribution to journalArticle

Stereocilia
Actins
Auditory Hair Cells
Actin Cytoskeleton
fascin
2012
13 Citations (Scopus)

Impacts of dystrophin and utrophin domains on actin structural dynamics: Implications for therapeutic design

Lin, A. Y., Prochniewicz-Nakayama, E., Henderson, D. M., Li, B., Ervasti, J. M. & Thomas, D. D., Jun 29 2012, In : Journal of Molecular Biology. 420, 1-2, p. 87-98 12 p.

Research output: Contribution to journalArticle

Utrophin
Dystrophin
Actins
Anisotropy
Genetic Therapy
117 Citations (Scopus)

Multi-isotope imaging mass spectrometry reveals slow protein turnover in hair-cell stereocilia

Zhang, D. S., Piazza, V., Perrin, B. J., Rzadzinska, A. K., Poczatek, J. C., Wang, M., Prosser, H. M., Ervasti, J. M., Corey, D. P. & Lechene, C. P., Jan 26 2012, In : Nature. 481, 7382, p. 520-524 5 p.

Research output: Contribution to journalArticle

Stereocilia
Isotopes
Hair
Mass Spectrometry
Actins
23 Citations (Scopus)

Restricted morphological and behavioral abnormalities following ablation of β-actin in the brain

Cheever, T. R., Li, B. & Ervasti, J. M., Mar 5 2012, In : PloS one. 7, 3, e32970.

Research output: Contribution to journalArticle

Ablation
actin
Actins
Brain
brain
12 Citations (Scopus)

The carboxy-terminal third of dystrophin enhances actin binding activity

Henderson, D. M., Lin, A. Y., Thomas, D. D. & Ervasti, J. M., Feb 24 2012, In : Journal of Molecular Biology. 416, 3, p. 414-424 11 p.

Research output: Contribution to journalArticle

Dystrophin
Actins
Microfilament Proteins
Anisotropy
Actin Cytoskeleton
2011
35 Citations (Scopus)

Axonal regeneration and neuronal function are preserved in motor neurons lacking ß-actin In Vivo

Cheever, T. R., Olson, E. A. & Ervasti, J. M., Mar 28 2011, In : PloS one. 6, 3, e17768.

Research output: Contribution to journalArticle

Motor Neurons
motor neurons
Neurons
actin
Actins
29 Citations (Scopus)

Internal deletion compromises the stability of dystrophin

Henderson, D. M., Belanto, J. J., Li, B., Heun-Johnson, H. & Ervasti, J. M., Aug 1 2011, In : Human molecular genetics. 20, 15, p. 2955-2963 9 p., ddr199.

Research output: Contribution to journalArticle

Dystrophin
Utrophin
Freezing
Proteins
Duchenne Muscular Dystrophy
18 Citations (Scopus)

Quadriceps myopathy caused by skeletal muscle-specific ablation of βcyto-actin

Prins, K. W., Call, J. A., Lowe, D. A. & Ervasti, J. M., Mar 15 2011, In : Journal of cell science. 124, 6, p. 951-957 7 p.

Research output: Contribution to journalArticle

Muscular Diseases
Dystrophin
Actins
Skeletal Muscle
Spectrin
10 Citations (Scopus)

Structural and functional properties of the actin gene family

Bunnell, T. M. & Ervasti, J. M., Jan 1 2011, In : Critical Reviews in Eukaryotic Gene Expression. 21, 3, p. 255-266 12 p.

Research output: Contribution to journalArticle

Actins
Protein Isoforms
Genes
Amino Acid Sequence Homology
Animal Models
18 Citations (Scopus)

TAT-μUtrophin mitigates the pathophysiology of dystrophin and utrophin double-knockout mice

Call, J. A., Ervasti, J. M. & Lowe, D. A., Jul 1 2011, In : Journal of Applied Physiology. 111, 1, p. 200-205 6 p.

Research output: Contribution to journalArticle

Utrophin
Inbred mdx Mouse
Dystrophin
Knockout Mice
Food
16 Citations (Scopus)

Transgenic overexpression of γ-cytoplasmic actin protects against eccentric contraction-induced force loss in mdx mice

Baltgalvis, K. A., Jaeger, M. A., Fitzsimons, D. P., Thayer, S. A., Lowe, D. A. & Ervasti, J. M., Oct 13 2011, In : Skeletal Muscle. 1, 1, 32.

Research output: Contribution to journalArticle

Inbred mdx Mouse
Actins
Skeletal Muscle
Calcium
Muscles
115 Citations (Scopus)

β-Actin specifically controls cell growth, migration, and the G-actin pool

Bunnell, T. M., Burbach, B. J., Shimizu, Y. & Ervasti, J. M., Nov 1 2011, In : Molecular biology of the cell. 22, 21, p. 4047-4058 12 p.

Research output: Contribution to journalArticle

Cell Movement
Actins
Growth
Fibroblasts
Knockout Mice
2010
44 Citations (Scopus)

Delayed embryonic development and impaired cell growth and survival in Actg1 null mice

Bunnell, T. M. & Ervasti, J. M., Sep 1 2010, In : Cytoskeleton. 67, 9, p. 564-572 9 p.

Research output: Contribution to journalArticle

Embryonic Development
Actins
Cell Survival
Growth
Growth Disorders
42 Citations (Scopus)
Dystrophin
Missense Mutation
Thermodynamics
Actins
Duchenne Muscular Dystrophy
70 Citations (Scopus)

Site mapping and characterization of O-glycan structures on α-dystroglycan isolated from rabbit skeletal muscle

Stalnaker, S. H., Hashmi, S., Lim, J. M., Aoki, K., Porterfield, M., Gutierrez-Sanchez, G., Wheeler, J., Ervasti, J. M., Bergmann, C., Tiemeyer, M. & Wells, L., Aug 6 2010, In : Journal of Biological Chemistry. 285, 32, p. 24882-24891 10 p.

Research output: Contribution to journalArticle

Dystroglycans
Tandem Mass Spectrometry
Glycosylation
Mass spectrometry
Polysaccharides
129 Citations (Scopus)

The actin gene family: Function follows isoform

Perrin, B. J. & Ervasti, J. M., Oct 1 2010, In : Cytoskeleton. 67, 10, p. 630-634 5 p.

Research output: Contribution to journalArticle

Actins
Protein Isoforms
Genes
Mammals
Amino Acid Sequence
51 Citations (Scopus)

β-Actin and γ-Actin are each dispensable for auditory hair cell development but required for stereocilia maintenance

Perrin, B. J., Sonnemann, K. J. & Ervasti, J. M., Oct 2010, In : PLoS Genetics. 6, 10, p. 1-12 12 p., e1001158.

Research output: Contribution to journalArticle

Auditory Hair Cells
Stereocilia
hair
hairs
actin
2009
25 Citations (Scopus)

Context-dependent functional substitution of α-skeletal actin by γ-cytoplasmic actin

Jaeger, M. A., Sonnemann, K. J., Fitzsimons, D. P., Prins, K. W. & Ervasti, J. M., Jul 1 2009, In : FASEB Journal. 23, 7, p. 2205-2214 10 p.

Research output: Contribution to journalArticle

Actins
Substitution reactions
Muscle
Muscles
Transgenic Mice
34 Citations (Scopus)

Dystrophin and utrophin have distinct effects on the structural dynamics of actin

Prochniewicz-Nakayama, E., Henderson, D., Ervasti, J. M. & Thomas, D. D., May 12 2009, In : Proceedings of the National Academy of Sciences of the United States of America. 106, 19, p. 7822-7827 6 p.

Research output: Contribution to journalArticle

Utrophin
Dystrophin
Actins
Anisotropy
Muscular Dystrophies
106 Citations (Scopus)

Dystrophin is a microtubule-associated protein

Prins, K. W., Humston, J. L., Mehta, A., Tate, V., Ralston, E. & Ervasti, J. M., Aug 10 2009, In : Journal of Cell Biology. 186, 3, p. 363-369 7 p.

Research output: Contribution to journalArticle

Dystrophin
Microtubule-Associated Proteins
Microtubules
Tubulin
Costameres
63 Citations (Scopus)

Functional substitution by TAT-utrophin in dystrophin-deficient mice

Sonnemann, K. J., Heun-Johnson, H., Turner, A. J., Baltgalvis, K. A., Lowe, D. A. & Ervasti, J. M., May 1 2009, In : PLoS Medicine. 6, 5, e1000083.

Research output: Contribution to journalArticle

Utrophin
Dystrophin
Inbred mdx Mouse
Phenotype
Proteins
97 Citations (Scopus)

γ-Actin is required for cytoskeletal maintenance but not development

Belyantseva, I. A., Perrin, B. J., Sonnemann, K. J., Zhu, M., Stepanyan, R., McGee, J., Frolenkov, G. I., Walsh, E. J., Friderici, K. H., Friedman, T. B. & Ervasti, J. M., Jun 16 2009, In : Proceedings of the National Academy of Sciences of the United States of America. 106, 24, p. 9703-9708 6 p.

Research output: Contribution to journalArticle

Actins
Maintenance
Stereocilia
Cytoskeleton
Hearing