Myostatin inhibition using mrk35 produces skeletal muscle growth and tubular aggregate formation in wild type and TgACTA1D286G nemaline myopathy mice

Research output: Contribution to journal › Journal article › Research › peer-review

Standard

Myostatin inhibition using mrk35 produces skeletal muscle growth and tubular aggregate formation in wild type and TgACTA1D286G nemaline myopathy mice. / Tinklenberg, Jennifer A.; Siebers, Emily M.; Beatka, Margaret J.; Meng, Hui; Yang, Lin; Zhang, Zizhao; Ross, Jacob A.; Ochala, Julien; Morris, Carl; Owens, Jane M.; Laing, Nigel G.; Nowak, Kristen J.; Lawlor, Michael W.

In: Human Molecular Genetics, Vol. 27, No. 4, ddx431, 15.02.2018, p. 638-648.

Research output: Contribution to journal › Journal article › Research › peer-review

Harvard

Tinklenberg, JA, Siebers, EM, Beatka, MJ, Meng, H, Yang, L, Zhang, Z, Ross, JA, Ochala, J, Morris, C, Owens, JM, Laing, NG, Nowak, KJ & Lawlor, MW 2018, 'Myostatin inhibition using mrk35 produces skeletal muscle growth and tubular aggregate formation in wild type and TgACTA1D286G nemaline myopathy mice', Human Molecular Genetics, vol. 27, no. 4, ddx431, pp. 638-648. https://doi.org/10.1093/hmg/ddx431

APA

Tinklenberg, J. A., Siebers, E. M., Beatka, M. J., Meng, H., Yang, L., Zhang, Z., Ross, J. A., Ochala, J., Morris, C., Owens, J. M., Laing, N. G., Nowak, K. J., & Lawlor, M. W. (2018). Myostatin inhibition using mrk35 produces skeletal muscle growth and tubular aggregate formation in wild type and TgACTA1D286G nemaline myopathy mice. Human Molecular Genetics, 27(4), 638-648. [ddx431]. https://doi.org/10.1093/hmg/ddx431

Vancouver

Tinklenberg JA, Siebers EM, Beatka MJ, Meng H, Yang L, Zhang Z et al. Myostatin inhibition using mrk35 produces skeletal muscle growth and tubular aggregate formation in wild type and TgACTA1D286G nemaline myopathy mice. Human Molecular Genetics. 2018 Feb 15;27(4):638-648. ddx431. https://doi.org/10.1093/hmg/ddx431

Author

Tinklenberg, Jennifer A. ; Siebers, Emily M. ; Beatka, Margaret J. ; Meng, Hui ; Yang, Lin ; Zhang, Zizhao ; Ross, Jacob A. ; Ochala, Julien ; Morris, Carl ; Owens, Jane M. ; Laing, Nigel G. ; Nowak, Kristen J. ; Lawlor, Michael W. / Myostatin inhibition using mrk35 produces skeletal muscle growth and tubular aggregate formation in wild type and TgACTA1D286G nemaline myopathy mice. In: Human Molecular Genetics. 2018 ; Vol. 27, No. 4. pp. 638-648.

Bibtex

@article{d68d05e6785c429cbb407561ae6c8f71,
title = "Myostatin inhibition using mrk35 produces skeletal muscle growth and tubular aggregate formation in wild type and TgACTA1D286G nemaline myopathy mice",
abstract = "Nemaline myopathy (NM) is a heterogeneous congenital skeletal muscle disease with cytoplasmic rod-like structures (nemaline bodies) in muscle tissue. While weakness in NM is related to contractile abnormalities, myofiber smallness is an additional abnormality in NM that may be treatable. We evaluated the effects of mRK35 (a myostatin inhibitor developed by Pfizer) treatment in the TgACTA1D286G mouse model of NM. mRK35 induced skeletal muscle growth that led to significant increases in animal bodyweight, forelimb grip strength and muscle fiber force, although it should be noted that animal weight and forelimb grip strength in untreated TgACTA1D286G mice was not different from controls. Treatment was also associated with an increase in the number of tubular aggregates found in skeletal muscle. These findings suggest that myostatin inhibition may be useful in promoting muscle growth and strength in Acta1-mutant muscle, while also further establishing the relationship between low levels of myostatin and tubular aggregate formation.",
author = "Tinklenberg, {Jennifer A.} and Siebers, {Emily M.} and Beatka, {Margaret J.} and Hui Meng and Lin Yang and Zizhao Zhang and Ross, {Jacob A.} and Julien Ochala and Carl Morris and Owens, {Jane M.} and Laing, {Nigel G.} and Nowak, {Kristen J.} and Lawlor, {Michael W.}",
year = "2018",
month = feb,
day = "15",
doi = "10.1093/hmg/ddx431",
language = "English",
volume = "27",
pages = "638--648",
journal = "Human Molecular Genetics",
issn = "0964-6906",
publisher = "Oxford University Press",
number = "4",

}

RIS

TY - JOUR

T1 - Myostatin inhibition using mrk35 produces skeletal muscle growth and tubular aggregate formation in wild type and TgACTA1D286G nemaline myopathy mice

AU - Tinklenberg, Jennifer A.

AU - Siebers, Emily M.

AU - Beatka, Margaret J.

AU - Meng, Hui

AU - Yang, Lin

AU - Zhang, Zizhao

AU - Ross, Jacob A.

AU - Ochala, Julien

AU - Morris, Carl

AU - Owens, Jane M.

AU - Laing, Nigel G.

AU - Nowak, Kristen J.

AU - Lawlor, Michael W.

PY - 2018/2/15

Y1 - 2018/2/15

N2 - Nemaline myopathy (NM) is a heterogeneous congenital skeletal muscle disease with cytoplasmic rod-like structures (nemaline bodies) in muscle tissue. While weakness in NM is related to contractile abnormalities, myofiber smallness is an additional abnormality in NM that may be treatable. We evaluated the effects of mRK35 (a myostatin inhibitor developed by Pfizer) treatment in the TgACTA1D286G mouse model of NM. mRK35 induced skeletal muscle growth that led to significant increases in animal bodyweight, forelimb grip strength and muscle fiber force, although it should be noted that animal weight and forelimb grip strength in untreated TgACTA1D286G mice was not different from controls. Treatment was also associated with an increase in the number of tubular aggregates found in skeletal muscle. These findings suggest that myostatin inhibition may be useful in promoting muscle growth and strength in Acta1-mutant muscle, while also further establishing the relationship between low levels of myostatin and tubular aggregate formation.

AB - Nemaline myopathy (NM) is a heterogeneous congenital skeletal muscle disease with cytoplasmic rod-like structures (nemaline bodies) in muscle tissue. While weakness in NM is related to contractile abnormalities, myofiber smallness is an additional abnormality in NM that may be treatable. We evaluated the effects of mRK35 (a myostatin inhibitor developed by Pfizer) treatment in the TgACTA1D286G mouse model of NM. mRK35 induced skeletal muscle growth that led to significant increases in animal bodyweight, forelimb grip strength and muscle fiber force, although it should be noted that animal weight and forelimb grip strength in untreated TgACTA1D286G mice was not different from controls. Treatment was also associated with an increase in the number of tubular aggregates found in skeletal muscle. These findings suggest that myostatin inhibition may be useful in promoting muscle growth and strength in Acta1-mutant muscle, while also further establishing the relationship between low levels of myostatin and tubular aggregate formation.

UR - http://www.scopus.com/inward/record.url?scp=85041501214&partnerID=8YFLogxK

U2 - 10.1093/hmg/ddx431

DO - 10.1093/hmg/ddx431

M3 - Journal article

C2 - 29293963

AN - SCOPUS:85041501214

VL - 27

SP - 638

EP - 648

JO - Human Molecular Genetics

JF - Human Molecular Genetics

SN - 0964-6906

IS - 4

M1 - ddx431

ER -

ID: 245662065