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Items: 1 to 50 of 55

1.

Effect of PGC1-beta ablation on myonuclear organisation.

Beedour R, Ross JA, Levy Y, Ochala J.

J Muscle Res Cell Motil. 2019 Sep 5. doi: 10.1007/s10974-019-09549-3. [Epub ahead of print]

PMID:
31485877
2.

Impairments in contractility and cytoskeletal organisation cause nuclear defects in nemaline myopathy.

Ross JA, Levy Y, Ripolone M, Kolb JS, Turmaine M, Holt M, Lindqvist J, Claeys KG, Weis J, Monforte M, Tasca G, Moggio M, Figeac N, Zammit PS, Jungbluth H, Fiorillo C, Vissing J, Witting N, Granzier H, Zanoteli E, Hardeman EC, Wallgren-Pettersson C, Ochala J.

Acta Neuropathol. 2019 Sep;138(3):477-495. doi: 10.1007/s00401-019-02034-8. Epub 2019 Jun 19.

3.

Molecular Consequences of the Myopathy-Related D286G Mutation on Actin Function.

Fan J, Chan C, McNamara EL, Nowak KJ, Iwamoto H, Ochala J.

Front Physiol. 2018 Dec 4;9:1756. doi: 10.3389/fphys.2018.01756. eCollection 2018.

4.

Reducing dynamin 2 (DNM2) rescues DNM2-related dominant centronuclear myopathy.

Buono S, Ross JA, Tasfaout H, Levy Y, Kretz C, Tayefeh L, Matson J, Guo S, Kessler P, Monia BP, Bitoun M, Ochala J, Laporte J, Cowling BS.

Proc Natl Acad Sci U S A. 2018 Oct 23;115(43):11066-11071. doi: 10.1073/pnas.1808170115. Epub 2018 Oct 5.

5.

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

Levy Y, Ross JA, Niglas M, Snetkov VA, Lynham S, Liao CY, Puckelwartz MJ, Hsu YM, McNally EM, Alsheimer M, Harridge SD, Young SG, Fong LG, Español Y, Lopez-Otin C, Kennedy BK, Lowe DA, Ochala J.

JCI Insight. 2018 Oct 4;3(19). pii: 120920. doi: 10.1172/jci.insight.120920.

6.

Defining the contribution of skeletal muscle pyruvate dehydrogenase α1 to exercise performance and insulin action.

Svensson K, Dent JR, Tahvilian S, Martins VF, Sathe A, Ochala J, Patel MS, Schenk S.

Am J Physiol Endocrinol Metab. 2018 Nov 1;315(5):E1034-E1045. doi: 10.1152/ajpendo.00241.2018. Epub 2018 Aug 28.

PMID:
30153068
7.

SIRT1 regulates nuclear number and domain size in skeletal muscle fibers.

Ross JA, Levy Y, Svensson K, Philp A, Schenk S, Ochala J.

J Cell Physiol. 2018 Sep;233(9):7157-7163. doi: 10.1002/jcp.26542. Epub 2018 Mar 25.

8.

Congenital myopathies: disorders of excitation-contraction coupling and muscle contraction.

Jungbluth H, Treves S, Zorzato F, Sarkozy A, Ochala J, Sewry C, Phadke R, Gautel M, Muntoni F.

Nat Rev Neurol. 2018 Mar;14(3):151-167. doi: 10.1038/nrneurol.2017.191. Epub 2018 Feb 2. Review.

PMID:
29391587
9.

Myostatin inhibition using mRK35 produces skeletal muscle growth and tubular aggregate formation in wild type and TgACTA1D286G nemaline myopathy mice.

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.

Hum Mol Genet. 2018 Feb 15;27(4):638-648. doi: 10.1093/hmg/ddx431.

10.

Exploring the Role of PGC-1α in Defining Nuclear Organisation in Skeletal Muscle Fibres.

Ross JA, Pearson A, Levy Y, Cardel B, Handschin C, Ochala J.

J Cell Physiol. 2017 Jun;232(6):1270-1274. doi: 10.1002/jcp.25678. Epub 2016 Dec 29.

11.

Current and future therapeutic approaches to the congenital myopathies.

Jungbluth H, Ochala J, Treves S, Gautel M.

Semin Cell Dev Biol. 2017 Apr;64:191-200. doi: 10.1016/j.semcdb.2016.08.004. Epub 2016 Aug 8. Review.

PMID:
27515125
12.

Dystrophin restoration therapy improves both the reduced excitability and the force drop induced by lengthening contractions in dystrophic mdx skeletal muscle.

Roy P, Rau F, Ochala J, Messéant J, Fraysse B, Lainé J, Agbulut O, Butler-Browne G, Furling D, Ferry A.

Skelet Muscle. 2016 Jul 20;6:23. doi: 10.1186/s13395-016-0096-4. eCollection 2016.

13.

Novel myosin-based therapies for congenital cardiac and skeletal myopathies.

Ochala J, Sun YB.

J Med Genet. 2016 Oct;53(10):651-4. doi: 10.1136/jmedgenet-2016-103881. Epub 2016 Jul 13. Review.

14.

Myopathy-inducing mutation H40Y in ACTA1 hampers actin filament structure and function.

Chan C, Fan J, Messer AE, Marston SB, Iwamoto H, Ochala J.

Biochim Biophys Acta. 2016 Aug;1862(8):1453-8. doi: 10.1016/j.bbadis.2016.04.013. Epub 2016 Apr 22.

15.

Modulating myosin restores muscle function in a mouse model of nemaline myopathy.

Lindqvist J, Levy Y, Pati-Alam A, Hardeman EC, Gregorevic P, Ochala J.

Ann Neurol. 2016 May;79(5):717-725. doi: 10.1002/ana.24619. Epub 2016 Mar 22.

16.

Tropomodulin 1 directly controls thin filament length in both wild-type and tropomodulin 4-deficient skeletal muscle.

Gokhin DS, Ochala J, Domenighetti AA, Fowler VM.

Development. 2015 Dec 15;142(24):4351-62. doi: 10.1242/dev.129171. Epub 2015 Nov 19.

17.

Ryanodine receptor fragmentation and sarcoplasmic reticulum Ca2+ leak after one session of high-intensity interval exercise.

Place N, Ivarsson N, Venckunas T, Neyroud D, Brazaitis M, Cheng AJ, Ochala J, Kamandulis S, Girard S, Volungevičius G, Paužas H, Mekideche A, Kayser B, Martinez-Redondo V, Ruas JL, Bruton J, Truffert A, Lanner JT, Skurvydas A, Westerblad H.

Proc Natl Acad Sci U S A. 2015 Dec 15;112(50):15492-7. doi: 10.1073/pnas.1507176112. Epub 2015 Nov 2.

18.

X-ray recordings reveal how a human disease-linked skeletal muscle α-actin mutation leads to contractile dysfunction.

Ochala J, Ravenscroft G, McNamara E, Nowak KJ, Iwamoto H.

J Struct Biol. 2015 Dec;192(3):331-335. doi: 10.1016/j.jsb.2015.09.008. Epub 2015 Sep 25.

PMID:
26407659
19.

Aberrant post-translational modifications compromise human myosin motor function in old age.

Li M, Ogilvie H, Ochala J, Artemenko K, Iwamoto H, Yagi N, Bergquist J, Larsson L.

Aging Cell. 2015 Apr;14(2):228-35. doi: 10.1111/acel.12307. Epub 2015 Feb 2.

20.

Skeletal muscle: a brief review of structure and function.

Frontera WR, Ochala J.

Calcif Tissue Int. 2015 Mar;96(3):183-95. doi: 10.1007/s00223-014-9915-y. Epub 2014 Oct 8. Review.

PMID:
25294644
21.

Sexually dimorphic myofilament function in a mouse model of nemaline myopathy.

Lindqvist J, Hardeman EC, Ochala J.

Arch Biochem Biophys. 2014 Dec 15;564:37-42. doi: 10.1016/j.abb.2014.09.011. Epub 2014 Sep 24.

PMID:
25261348
22.

Pointed-end capping by tropomodulin modulates actomyosin crossbridge formation in skeletal muscle fibers.

Ochala J, Gokhin DS, Iwamoto H, Fowler VM.

FASEB J. 2014 Jan;28(1):408-15. doi: 10.1096/fj.13-239640. Epub 2013 Sep 26.

23.

Skeletal and cardiac α-actin isoforms differently modulate myosin cross-bridge formation and myofibre force production.

Ochala J, Iwamoto H, Ravenscroft G, Laing NG, Nowak KJ.

Hum Mol Genet. 2013 Nov 1;22(21):4398-404. doi: 10.1093/hmg/ddt289. Epub 2013 Jun 19.

24.

Myofilament lattice structure in presence of a skeletal myopathy-related tropomyosin mutation.

Ochala J, Iwamoto H.

J Muscle Res Cell Motil. 2013 Aug;34(3-4):171-5. doi: 10.1007/s10974-013-9345-x. Epub 2013 May 18.

PMID:
23686574
25.

Distinct underlying mechanisms of limb and respiratory muscle fiber weaknesses in nemaline myopathy.

Lindqvist J, Cheng AJ, Renaud G, Hardeman EC, Ochala J.

J Neuropathol Exp Neurol. 2013 Jun;72(6):472-81. doi: 10.1097/NEN.0b013e318293b1cc.

PMID:
23656990
26.

The fraction of strongly bound cross-bridges is increased in mice that carry the myopathy-linked myosin heavy chain mutation MYH4L342Q.

Lindqvist J, Iwamoto H, Blanco G, Ochala J.

Dis Model Mech. 2013 May;6(3):834-40. doi: 10.1242/dmm.011155. Epub 2013 Jan 18.

27.

Sparing of muscle mass and function by passive loading in an experimental intensive care unit model.

Renaud G, Llano-Diez M, Ravara B, Gorza L, Feng HZ, Jin JP, Cacciani N, Gustafson AM, Ochala J, Corpeno R, Li M, Hedström Y, Ford GC, Nair KS, Larsson L.

J Physiol. 2013 Mar 1;591(5):1385-402. doi: 10.1113/jphysiol.2012.248724. Epub 2012 Dec 24.

28.

Nemaline myopathy-related skeletal muscle α-actin (ACTA1) mutation, Asp286Gly, prevents proper strong myosin binding and triggers muscle weakness.

Ochala J, Ravenscroft G, Laing NG, Nowak KJ.

PLoS One. 2012;7(9):e45923. doi: 10.1371/journal.pone.0045923. Epub 2012 Sep 20.

29.

Congenital myopathy-causing tropomyosin mutations induce thin filament dysfunction via distinct physiological mechanisms.

Ochala J, Gokhin DS, Pénisson-Besnier I, Quijano-Roy S, Monnier N, Lunardi J, Romero NB, Fowler VM.

Hum Mol Genet. 2012 Oct 15;21(20):4473-85. Epub 2012 Jul 13.

30.

Impaired adaptive response to mechanical overloading in dystrophic skeletal muscle.

Joanne P, Hourdé C, Ochala J, Caudéran Y, Medja F, Vignaud A, Mouisel E, Hadj-Said W, Arandel L, Garcia L, Goyenvalle A, Mounier R, Zibroba D, Sakamoto K, Butler-Browne G, Agbulut O, Ferry A.

PLoS One. 2012;7(4):e35346. doi: 10.1371/journal.pone.0035346. Epub 2012 Apr 12. Erratum in: PLoS One. 2013;8(6). doi:10.1371/annotation/2c0f8405-a2cc-4240-8e1b-117d3ac1a154. Sakamato, Kei [name corrected to Sakamoto, Kei].

31.

A myopathy-related actin mutation increases contractile function.

Lindqvist J, Pénisson-Besnier I, Iwamoto H, Li M, Yagi N, Ochala J.

Acta Neuropathol. 2012 May;123(5):739-46. doi: 10.1007/s00401-012-0962-z. Epub 2012 Feb 23.

PMID:
22358459
32.

Myofibrillar myopathy caused by a mutation in the motor domain of mouse MyHC IIb.

Kurapati R, McKenna C, Lindqvist J, Williams D, Simon M, LeProust E, Baker J, Cheeseman M, Carroll N, Denny P, Laval S, Lochmüller H, Ochala J, Blanco G.

Hum Mol Genet. 2012 Apr 15;21(8):1706-24. doi: 10.1093/hmg/ddr605. Epub 2011 Dec 23.

PMID:
22199023
33.

Mechanisms underlying the sparing of masticatory versus limb muscle function in an experimental critical illness model.

Aare S, Ochala J, Norman HS, Radell P, Eriksson LI, Göransson H, Chen YW, Hoffman EP, Larsson L.

Physiol Genomics. 2011 Dec 16;43(24):1334-50. doi: 10.1152/physiolgenomics.00116.2011. Epub 2011 Oct 18.

PMID:
22010006
34.

Diaphragm muscle weakness in an experimental porcine intensive care unit model.

Ochala J, Renaud G, Llano Diez M, Banduseela VC, Aare S, Ahlbeck K, Radell PJ, Eriksson LI, Larsson L.

PLoS One. 2011;6(6):e20558. doi: 10.1371/journal.pone.0020558. Epub 2011 Jun 15.

35.

Factors underlying the early limb muscle weakness in acute quadriplegic myopathy using an experimental ICU porcine model.

Ochala J, Ahlbeck K, Radell PJ, Eriksson LI, Larsson L.

PLoS One. 2011;6(6):e20876. doi: 10.1371/journal.pone.0020876. Epub 2011 Jun 14.

36.

Disrupted myosin cross-bridge cycling kinetics triggers muscle weakness in nebulin-related myopathy.

Ochala J, Lehtokari VL, Iwamoto H, Li M, Feng HZ, Jin JP, Yagi N, Wallgren-Pettersson C, Pénisson-Besnier I, Larsson L.

FASEB J. 2011 Jun;25(6):1903-13. doi: 10.1096/fj.10-176727. Epub 2011 Feb 24.

37.

Preferential skeletal muscle myosin loss in response to mechanical silencing in a novel rat intensive care unit model: underlying mechanisms.

Ochala J, Gustafson AM, Diez ML, Renaud G, Li M, Aare S, Qaisar R, Banduseela VC, Hedström Y, Tang X, Dworkin B, Ford GC, Nair KS, Perera S, Gautel M, Larsson L.

J Physiol. 2011 Apr 15;589(Pt 8):2007-26. doi: 10.1113/jphysiol.2010.202044. Epub 2011 Feb 14.

38.

Relationship between force and size in human single muscle fibres.

Krivickas LS, Dorer DJ, Ochala J, Frontera WR.

Exp Physiol. 2011 May;96(5):539-47. doi: 10.1113/expphysiol.2010.055269. Epub 2011 Feb 11.

39.

A myopathy-linked tropomyosin mutation severely alters thin filament conformational changes during activation.

Ochala J, Iwamoto H, Larsson L, Yagi N.

Proc Natl Acad Sci U S A. 2010 May 25;107(21):9807-12. doi: 10.1073/pnas.1001733107. Epub 2010 May 10.

40.

Ca2+ sensitizers: An emerging class of agents for counterbalancing weakness in skeletal muscle diseases?

Ochala J.

Neuromuscul Disord. 2010 Feb;20(2):98-101. doi: 10.1016/j.nmd.2009.11.010. Epub 2009 Dec 16. Review.

PMID:
20006502
41.

Maintenance of muscle mass, fiber size, and contractile function in mice lacking the Z-disc protein myotilin.

Ochala J, Carpén O, Larsson L.

Ups J Med Sci. 2009;114(4):235-41. doi: 10.3109/03009730903276399.

42.

EMD 57033 partially reverses ventilator-induced diaphragm muscle fibre calcium desensitisation.

Ochala J, Radell PJ, Eriksson LI, Larsson L.

Pflugers Arch. 2010 Feb;459(3):475-83. doi: 10.1007/s00424-009-0744-1. Epub 2009 Oct 2.

PMID:
19798510
43.

Gene expression and muscle fiber function in a porcine ICU model.

Banduseela VC, Ochala J, Chen YW, Göransson H, Norman H, Radell P, Eriksson LI, Hoffman EP, Larsson L.

Physiol Genomics. 2009 Nov 6;39(3):141-59. doi: 10.1152/physiolgenomics.00026.2009. Epub 2009 Aug 25.

PMID:
19706692
44.

Muscle paralysis and myosin loss in a patient with cancer cachexia.

Banduseela V, Ochala J, Lamberg K, Kalimo H, Larsson L.

Acta Myol. 2007 Dec;26(3):136-44.

45.

Thin filament proteins mutations associated with skeletal myopathies: defective regulation of muscle contraction.

Ochala J.

J Mol Med (Berl). 2008 Nov;86(11):1197-204. doi: 10.1007/s00109-008-0380-9. Epub 2008 Jun 24. Review.

PMID:
18574571
46.

Defective regulation of contractile function in muscle fibres carrying an E41K beta-tropomyosin mutation.

Ochala J, Li M, Ohlsson M, Oldfors A, Larsson L.

J Physiol. 2008 Jun 15;586(12):2993-3004. doi: 10.1113/jphysiol.2008.153650. Epub 2008 Apr 17.

47.

Effects of a preferential myosin loss on Ca2+ activation of force generation in single human skeletal muscle fibres.

Ochala J, Larsson L.

Exp Physiol. 2008 Apr;93(4):486-95. doi: 10.1113/expphysiol.2007.041798. Epub 2008 Feb 1.

48.

Single skeletal muscle fiber elastic and contractile characteristics in young and older men.

Ochala J, Frontera WR, Dorer DJ, Van Hoecke J, Krivickas LS.

J Gerontol A Biol Sci Med Sci. 2007 Apr;62(4):375-81.

PMID:
17452730
49.

Effects of a R133W beta-tropomyosin mutation on regulation of muscle contraction in single human muscle fibres.

Ochala J, Li M, Tajsharghi H, Kimber E, Tulinius M, Oldfors A, Larsson L.

J Physiol. 2007 Jun 15;581(Pt 3):1283-92. Epub 2007 Apr 12.

50.

Changes in muscle and joint elasticity following long-term strength training in old age.

Ochala J, Lambertz D, Van Hoecke J, Pousson M.

Eur J Appl Physiol. 2007 Jul;100(5):491-8. Epub 2006 May 10.

PMID:
16685553

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