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Prelamin A causes aberrant myonuclear arrangement and results in muscle fiber weakness

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Prelamin A causes aberrant myonuclear arrangement and results in muscle fiber weakness. / Levy, Yotam; Ross, Jacob A.; Niglas, Marili; Snetkov, Vladimir; Lynham, Steven ; Liao, Chen-Yu; Puckelwartz, Megan; Hsu, Yueh-Mei; McNally, Elizabeth; Alsheimer, Manfred; Harridge, Stephen D. R. ; Young, Stephen G.; Fong, Loren G.; Espanol, Yaiza; Lopez-Otin, Carlos; Kennedy, Brian K.; Lowe, Dawn A.; Ochala, Julien.

In: Journal of Clinical Investigation Insight, Vol. 3, No. 19, e120920, 04.10.2018.

Research output: Contribution to journalArticle

Harvard

Levy, Y, Ross, JA, Niglas, M, Snetkov, V, Lynham, S, Liao, C-Y, Puckelwartz, M, Hsu, Y-M, McNally, E, Alsheimer, M, Harridge, SDR, Young, SG, Fong, LG, Espanol, Y, Lopez-Otin, C, Kennedy, BK, Lowe, DA & Ochala, J 2018, 'Prelamin A causes aberrant myonuclear arrangement and results in muscle fiber weakness', Journal of Clinical Investigation Insight, vol. 3, no. 19, e120920. https://doi.org/10.1172/jci.insight.120920

APA

Levy, Y., Ross, J. A., Niglas, M., Snetkov, V., Lynham, S., Liao, C-Y., Puckelwartz, M., Hsu, Y-M., McNally, E., Alsheimer, M., Harridge, S. D. R., Young, S. G., Fong, L. G., Espanol, Y., Lopez-Otin, C., Kennedy, B. K., Lowe, D. A., & Ochala, J. (2018). Prelamin A causes aberrant myonuclear arrangement and results in muscle fiber weakness. Journal of Clinical Investigation Insight, 3(19), [e120920]. https://doi.org/10.1172/jci.insight.120920

Vancouver

Levy Y, Ross JA, Niglas M, Snetkov V, Lynham S, Liao C-Y et al. Prelamin A causes aberrant myonuclear arrangement and results in muscle fiber weakness. Journal of Clinical Investigation Insight. 2018 Oct 4;3(19). e120920. https://doi.org/10.1172/jci.insight.120920

Author

Levy, Yotam ; Ross, Jacob A. ; Niglas, Marili ; Snetkov, Vladimir ; Lynham, Steven ; Liao, Chen-Yu ; Puckelwartz, Megan ; Hsu, Yueh-Mei ; McNally, Elizabeth ; Alsheimer, Manfred ; Harridge, Stephen D. R. ; Young, Stephen G. ; Fong, Loren G. ; Espanol, Yaiza ; Lopez-Otin, Carlos ; Kennedy, Brian K. ; Lowe, Dawn A. ; Ochala, Julien. / Prelamin A causes aberrant myonuclear arrangement and results in muscle fiber weakness. In: Journal of Clinical Investigation Insight. 2018 ; Vol. 3, No. 19.

Bibtex Download

@article{1908d0497e1347618c21c1b897ea6f99,
title = "Prelamin A causes aberrant myonuclear arrangement and results in muscle fiber weakness",
abstract = "Physiological and premature aging are frequently associated with an accumulation of prelamin A, a precursor of lamin A, in the nuclear envelope of various cell types. Here, we aimed to underpin the hitherto unknown mechanisms by which prelamin A alters myonuclear organization and muscle fiber function. By experimentally studying membrane-permeabilized myofibers from various transgenic mouse lines, our results indicate that, in the presence of prelamin A, the abundance of nuclei and myosin content is markedly reduced within muscle fibers. This leads to a concept by which the remaining myonuclei are very distant from each other and are pushed to function beyond their maximum cytoplasmic capacity, ultimately inducing muscle fiber weakness.",
author = "Yotam Levy and Ross, {Jacob A.} and Marili Niglas and Vladimir Snetkov and Steven Lynham and Chen-Yu Liao and Megan Puckelwartz and Yueh-Mei Hsu and Elizabeth McNally and Manfred Alsheimer and Harridge, {Stephen D. R.} and Young, {Stephen G.} and Fong, {Loren G.} and Yaiza Espanol and Carlos Lopez-Otin and Kennedy, {Brian K.} and Lowe, {Dawn A.} and Julien Ochala",
year = "2018",
month = oct,
day = "4",
doi = "10.1172/jci.insight.120920",
language = "English",
volume = "3",
journal = "Journal of Clinical Investigation Insight",
number = "19",

}

RIS (suitable for import to EndNote) Download

TY - JOUR

T1 - Prelamin A causes aberrant myonuclear arrangement and results in muscle fiber weakness

AU - Levy, Yotam

AU - Ross, Jacob A.

AU - Niglas, Marili

AU - Snetkov, Vladimir

AU - Lynham, Steven

AU - Liao, Chen-Yu

AU - Puckelwartz, Megan

AU - Hsu, Yueh-Mei

AU - McNally, Elizabeth

AU - Alsheimer, Manfred

AU - Harridge, Stephen D. R.

AU - Young, Stephen G.

AU - Fong, Loren G.

AU - Espanol, Yaiza

AU - Lopez-Otin, Carlos

AU - Kennedy, Brian K.

AU - Lowe, Dawn A.

AU - Ochala, Julien

PY - 2018/10/4

Y1 - 2018/10/4

N2 - Physiological and premature aging are frequently associated with an accumulation of prelamin A, a precursor of lamin A, in the nuclear envelope of various cell types. Here, we aimed to underpin the hitherto unknown mechanisms by which prelamin A alters myonuclear organization and muscle fiber function. By experimentally studying membrane-permeabilized myofibers from various transgenic mouse lines, our results indicate that, in the presence of prelamin A, the abundance of nuclei and myosin content is markedly reduced within muscle fibers. This leads to a concept by which the remaining myonuclei are very distant from each other and are pushed to function beyond their maximum cytoplasmic capacity, ultimately inducing muscle fiber weakness.

AB - Physiological and premature aging are frequently associated with an accumulation of prelamin A, a precursor of lamin A, in the nuclear envelope of various cell types. Here, we aimed to underpin the hitherto unknown mechanisms by which prelamin A alters myonuclear organization and muscle fiber function. By experimentally studying membrane-permeabilized myofibers from various transgenic mouse lines, our results indicate that, in the presence of prelamin A, the abundance of nuclei and myosin content is markedly reduced within muscle fibers. This leads to a concept by which the remaining myonuclei are very distant from each other and are pushed to function beyond their maximum cytoplasmic capacity, ultimately inducing muscle fiber weakness.

U2 - 10.1172/jci.insight.120920

DO - 10.1172/jci.insight.120920

M3 - Article

VL - 3

JO - Journal of Clinical Investigation Insight

JF - Journal of Clinical Investigation Insight

IS - 19

M1 - e120920

ER -

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