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Items: 1 to 50 of 237

1.

KBTBD13 is an actin-binding protein that modulates muscle kinetics.

de Winter JM, Molenaar JP, Yuen M, van der Pijl R, Shen S, Conijn S, van de Locht M, Willigenburg M, Bogaards SJ, van Kleef ES, Lassche S, Persson M, Rassier DE, Sztal TE, Ruparelia AA, Oorschot V, Ramm G, Hall TE, Xiong Z, Johnson CN, Li F, Kiss B, Lozano-Vidal N, Boon RA, Marabita M, Nogara L, Blaauw B, Rodenburg RJ, Kϋsters B, Doorduin J, Beggs AH, Granzier H, Campbell K, Ma W, Irving T, Malfatti E, Romero NB, Bryson-Richardson RJ, van Engelen BG, Voermans NC, Ottenheijm CA.

J Clin Invest. 2019 Oct 31. pii: 124000. doi: 10.1172/JCI124000. [Epub ahead of print]

2.

Functional Characterization of the Intact Diaphragm in a Nebulin-Based Nemaline Myopathy (NM) Model-Effects of the Fast Skeletal Muscle Troponin Activator tirasemtiv.

Lee EJ, Kolb J, Hwee DT, Malik FI, Granzier HL.

Int J Mol Sci. 2019 Oct 10;20(20). pii: E5008. doi: 10.3390/ijms20205008.

3.

Failure to identify modifiers of NEBULIN-related nemaline myopathy in two pre-clinical models of the disease.

Qiu B, Ruston J, Granzier H, Justice MJ, Dowling JJ.

Biol Open. 2019 Sep 18;8(9). pii: bio044867. doi: 10.1242/bio.044867.

4.

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.

5.

Fine mapping titin's C-zone: Matching cardiac myosin-binding protein C stripes with titin's super-repeats.

Tonino P, Kiss B, Gohlke J, Smith JE 3rd, Granzier H.

J Mol Cell Cardiol. 2019 Aug;133:47-56. doi: 10.1016/j.yjmcc.2019.05.026. Epub 2019 May 31.

6.

Osteopontin Promotes Left Ventricular Diastolic Dysfunction Through a Mitochondrial Pathway.

Yousefi K, Irion CI, Takeuchi LM, Ding W, Lambert G, Eisenberg T, Sukkar S, Granzier HL, Methawasin M, Lee DI, Hahn VS, Kass DA, Hatzistergos KE, Hare JM, Webster KA, Shehadeh LA.

J Am Coll Cardiol. 2019 Jun 4;73(21):2705-2718. doi: 10.1016/j.jacc.2019.02.074.

PMID:
31146816
7.

Diaphragm contractile weakness due to reduced mechanical loading: role of titin.

van der Pijl RJ, Granzier HL, Ottenheijm CAC.

Am J Physiol Cell Physiol. 2019 Aug 1;317(2):C167-C176. doi: 10.1152/ajpcell.00509.2018. Epub 2019 May 1.

PMID:
31042425
8.

Progress on the regulation of myofibrillar function: Part 2.

Granzier HL, Moss RL.

J Gen Physiol. 2019 May 6;151(5):609. doi: 10.1085/jgp.201912380. Epub 2019 Apr 15. No abstract available.

9.

Nebulin and titin modulate cross-bridge cycling and length-dependent calcium sensitivity.

Mijailovich SM, Stojanovic B, Nedic D, Svicevic M, Geeves MA, Irving TC, Granzier HL.

J Gen Physiol. 2019 May 6;151(5):680-704. doi: 10.1085/jgp.201812165. Epub 2019 Apr 4.

10.

Titin mutations and muscle disease.

Kellermayer D, Smith JE 3rd, Granzier H.

Pflugers Arch. 2019 May;471(5):673-682. doi: 10.1007/s00424-019-02272-5. Epub 2019 Mar 27. Review.

PMID:
30919088
11.

Response to: Thick Filament Length Changes in Muscle Have Both Elastic and Structural Components.

Ma W, Gong H, Kiss B, Lee EJ, Granzier H, Irving T.

Biophys J. 2019 Mar 19;116(6):985-986. doi: 10.1016/j.bpj.2019.02.010. Epub 2019 Mar 5. No abstract available.

PMID:
30850114
12.

A missense variant in the titin gene in Doberman pinscher dogs with familial dilated cardiomyopathy and sudden cardiac death.

Meurs KM, Friedenberg SG, Kolb J, Saripalli C, Tonino P, Woodruff K, Olby NJ, Keene BW, Adin DB, Yost OL, DeFrancesco TC, Lahmers S, Tou S, Shelton GD, Granzier H.

Hum Genet. 2019 May;138(5):515-524. doi: 10.1007/s00439-019-01973-2. Epub 2019 Feb 4.

PMID:
30715562
13.

Deleting Full Length Titin Versus the Titin M-Band Region Leads to Differential Mechanosignaling and Cardiac Phenotypes.

Radke MH, Polack C, Methawasin M, Fink C, Granzier HL, Gotthardt M.

Circulation. 2019 Apr 9;139(15):1813-1827. doi: 10.1161/CIRCULATIONAHA.118.037588.

PMID:
30700140
14.

Deleting nebulin's C-terminus reveals its importance to sarcomeric structure and function and is sufficient to invoke nemaline myopathy.

Li F, Barton ER, Granzier H.

Hum Mol Genet. 2019 May 15;28(10):1709-1725. doi: 10.1093/hmg/ddz016.

PMID:
30689900
15.

Myostatin Inhibition Using ActRIIB-mFc Does Not Produce Weight Gain or Strength in the Nebulin Conditional KO Mouse.

Tinklenberg JA, Siebers EM, Beatka MJ, Fickau BA, Ayres S, Meng H, Yang L, Simpson P, Granzier HL, Lawlor MW.

J Neuropathol Exp Neurol. 2019 Feb 1;78(2):130-139. doi: 10.1093/jnen/nly120.

PMID:
30597051
16.

Metformin improves diastolic function in an HFpEF-like mouse model by increasing titin compliance.

Slater RE, Strom JG, Methawasin M, Liss M, Gotthardt M, Sweitzer N, Granzier HL.

J Gen Physiol. 2019 Jan 7;151(1):42-52. doi: 10.1085/jgp.201812259. Epub 2018 Dec 19.

17.

Downsizing the molecular spring of the giant protein titin reveals that skeletal muscle titin determines passive stiffness and drives longitudinal hypertrophy.

Brynnel A, Hernandez Y, Kiss B, Lindqvist J, Adler M, Kolb J, van der Pijl R, Gohlke J, Strom J, Smith J, Ottenheijm C, Granzier HL.

Elife. 2018 Dec 19;7. pii: e40532. doi: 10.7554/eLife.40532.

18.

Sarcomere length-dependent effects on Ca2+-troponin regulation in myocardium expressing compliant titin.

Li KL, Methawasin M, Tanner BCW, Granzier HL, Solaro RJ, Dong WJ.

J Gen Physiol. 2019 Jan 7;151(1):30-41. doi: 10.1085/jgp.201812218. Epub 2018 Dec 6.

19.

Nebulin increases thin filament stiffness and force per cross-bridge in slow-twitch soleus muscle fibers.

Kawai M, Karam TS, Kolb J, Wang L, Granzier HL.

J Gen Physiol. 2018 Nov 5;150(11):1510-1522. doi: 10.1085/jgp.201812104. Epub 2018 Oct 9.

20.

Thick-Filament Extensibility in Intact Skeletal Muscle.

Ma W, Gong H, Kiss B, Lee EJ, Granzier H, Irving T.

Biophys J. 2018 Oct 16;115(8):1580-1588. doi: 10.1016/j.bpj.2018.08.038. Epub 2018 Sep 4.

21.

Nebulin stiffens the thin filament and augments cross-bridge interaction in skeletal muscle.

Kiss B, Lee EJ, Ma W, Li FW, Tonino P, Mijailovich SM, Irving TC, Granzier HL.

Proc Natl Acad Sci U S A. 2018 Oct 9;115(41):10369-10374. doi: 10.1073/pnas.1804726115. Epub 2018 Sep 24.

22.

Softening the Stressed Giant Titin in Diabetes Mellitus.

Methawasin M, Granzier H.

Circ Res. 2018 Jul 20;123(3):315-317. doi: 10.1161/CIRCRESAHA.118.313396. No abstract available.

23.

Titin-based mechanosensing modulates muscle hypertrophy.

van der Pijl R, Strom J, Conijn S, Lindqvist J, Labeit S, Granzier H, Ottenheijm C.

J Cachexia Sarcopenia Muscle. 2018 Oct;9(5):947-961. doi: 10.1002/jcsm.12319. Epub 2018 Jul 5.

24.

Congenital Titinopathy: Comprehensive characterization and pathogenic insights.

Oates EC, Jones KJ, Donkervoort S, Charlton A, Brammah S, Smith JE 3rd, Ware JS, Yau KS, Swanson LC, Whiffin N, Peduto AJ, Bournazos A, Waddell LB, Farrar MA, Sampaio HA, Teoh HL, Lamont PJ, Mowat D, Fitzsimons RB, Corbett AJ, Ryan MM, O'Grady GL, Sandaradura SA, Ghaoui R, Joshi H, Marshall JL, Nolan MA, Kaur S, Punetha J, Töpf A, Harris E, Bakshi M, Genetti CA, Marttila M, Werlauff U, Streichenberger N, Pestronk A, Mazanti I, Pinner JR, Vuillerot C, Grosmann C, Camacho A, Mohassel P, Leach ME, Foley AR, Bharucha-Goebel D, Collins J, Connolly AM, Gilbreath HR, Iannaccone ST, Castro D, Cummings BB, Webster RI, Lazaro L, Vissing J, Coppens S, Deconinck N, Luk HM, Thomas NH, Foulds NC, Illingworth MA, Ellard S, McLean CA, Phadke R, Ravenscroft G, Witting N, Hackman P, Richard I, Cooper ST, Kamsteeg EJ, Hoffman EP, Bushby K, Straub V, Udd B, Ferreiro A, North KN, Clarke NF, Lek M, Beggs AH, Bönnemann CG, MacArthur DG, Granzier H, Davis MR, Laing NG.

Ann Neurol. 2018 Jun;83(6):1105-1124. doi: 10.1002/ana.25241.

25.

Positive End-Expiratory Pressure Ventilation Induces Longitudinal Atrophy in Diaphragm Fibers.

Lindqvist J, van den Berg M, van der Pijl R, Hooijman PE, Beishuizen A, Elshof J, de Waard M, Girbes A, Spoelstra-de Man A, Shi ZH, van den Brom C, Bogaards S, Shen S, Strom J, Granzier H, Kole J, Musters RJP, Paul MA, Heunks LMA, Ottenheijm CAC.

Am J Respir Crit Care Med. 2018 Aug 15;198(4):472-485. doi: 10.1164/rccm.201709-1917OC.

26.

Histone deacetylase activity governs diastolic dysfunction through a nongenomic mechanism.

Jeong MY, Lin YH, Wennersten SA, Demos-Davies KM, Cavasin MA, Mahaffey JH, Monzani V, Saripalli C, Mascagni P, Reece TB, Ambardekar AV, Granzier HL, Dinarello CA, McKinsey TA.

Sci Transl Med. 2018 Feb 7;10(427). pii: eaao0144. doi: 10.1126/scitranslmed.aao0144.

27.

Correction to: The Sydney Heart Bank: improving translational research while eliminating or reducing the use of animal models of human heart disease.

Dos Remedios CG, Lal SP, Li A, McNamara J, Keogh A, Macdonald PS, Cooke R, Ehler E, Knöll R, Marston SB, Stelzer J, Granzier H, Bezzina C, van Dijk S, De Man F, Stienen GJM, Odeberg J, Pontén F, Linke WA, van der Velden J.

Biophys Rev. 2018 Jun;10(3):941. doi: 10.1007/s12551-018-0397-4.

28.

The giant protein titin regulates the length of the striated muscle thick filament.

Tonino P, Kiss B, Strom J, Methawasin M, Smith JE 3rd, Kolb J, Labeit S, Granzier H.

Nat Commun. 2017 Oct 19;8(1):1041. doi: 10.1038/s41467-017-01144-9.

29.

The Sydney Heart Bank: improving translational research while eliminating or reducing the use of animal models of human heart disease.

Dos Remedios CG, Lal SP, Li A, McNamara J, Keogh A, Macdonald PS, Cooke R, Ehler E, Knöll R, Marston SB, Stelzer J, Granzier H, Bezzina C, van Dijk S, De Man F, Stienen GJM, Odeberg J, Pontén F, Linke WA, Linke W, van der Velden J.

Biophys Rev. 2017 Aug;9(4):431-441. doi: 10.1007/s12551-017-0305-3. Epub 2017 Aug 14. Review. Erratum in: Biophys Rev. 2018 Feb 1;:. Linke W [corrected to Linke WA].

30.

Novex-3, the tiny titin of muscle.

Kellermayer D, Smith JE 3rd, Granzier H.

Biophys Rev. 2017 Jun;9(3):201-206. doi: 10.1007/s12551-017-0261-y. Epub 2017 Apr 7. Review.

31.

Effect of exercise on passive myocardial stiffness in mice with diastolic dysfunction.

Slater RE, Strom JG, Granzier H.

J Mol Cell Cardiol. 2017 Jul;108:24-33. doi: 10.1016/j.yjmcc.2017.04.006. Epub 2017 May 3.

PMID:
28476659
33.

Muscle weakness in respiratory and peripheral skeletal muscles in a mouse model for nebulin-based nemaline myopathy.

Joureau B, de Winter JM, Stam K, Granzier H, Ottenheijm CA.

Neuromuscul Disord. 2017 Jan;27(1):83-89. doi: 10.1016/j.nmd.2016.10.004. Epub 2016 Oct 25.

PMID:
27890461
34.

Reducing RBM20 activity improves diastolic dysfunction and cardiac atrophy.

Hinze F, Dieterich C, Radke MH, Granzier H, Gotthardt M.

J Mol Med (Berl). 2016 Dec;94(12):1349-1358. Epub 2016 Nov 26.

35.
36.

Exploration of pathomechanisms triggered by a single-nucleotide polymorphism in titin's I-band: the cardiomyopathy-linked mutation T2580I.

Bogomolovas J, Fleming JR, Anderson BR, Williams R, Lange S, Simon B, Khan MM, Rudolf R, Franke B, Bullard B, Rigden DJ, Granzier H, Labeit S, Mayans O.

Open Biol. 2016 Sep;6(9). pii: 160114.

37.

Increased Titin Compliance Reduced Length-Dependent Contraction and Slowed Cross-Bridge Kinetics in Skinned Myocardial Strips from Rbm (20ΔRRM) Mice.

Pulcastro HC, Awinda PO, Methawasin M, Granzier H, Dong W, Tanner BC.

Front Physiol. 2016 Jul 29;7:322. doi: 10.3389/fphys.2016.00322. eCollection 2016.

38.

A Review of the Giant Protein Titin in Clinical Molecular Diagnostics of Cardiomyopathies.

Gigli M, Begay RL, Morea G, Graw SL, Sinagra G, Taylor MR, Granzier H, Mestroni L.

Front Cardiovasc Med. 2016 Jul 21;3:21. doi: 10.3389/fcvm.2016.00021. eCollection 2016. Review.

39.

Alternative Splicing of Titin Restores Diastolic Function in an HFpEF-Like Genetic Murine Model (TtnΔIAjxn).

Bull M, Methawasin M, Strom J, Nair P, Hutchinson K, Granzier H.

Circ Res. 2016 Sep 2;119(6):764-72. doi: 10.1161/CIRCRESAHA.116.308904. Epub 2016 Jul 28.

40.

Exome sequencing reveals a nebulin nonsense mutation in a dog model of nemaline myopathy.

Evans JM, Cox ML, Huska J, Li F, Gaitero L, Guo LT, Casal ML, Granzier HL, Shelton GD, Clark LA.

Mamm Genome. 2016 Oct;27(9-10):495-502. doi: 10.1007/s00335-016-9644-9. Epub 2016 May 23.

41.

Sex dimorphisms of crossbridge cycling kinetics in transgenic hypertrophic cardiomyopathy mice.

Birch CL, Behunin SM, Lopez-Pier MA, Danilo C, Lipovka Y, Saripalli C, Granzier H, Konhilas JP.

Am J Physiol Heart Circ Physiol. 2016 Jul 1;311(1):H125-36. doi: 10.1152/ajpheart.00592.2015. Epub 2016 May 6.

42.

Thin filament length in the cardiac sarcomere varies with sarcomere length but is independent of titin and nebulin.

Kolb J, Li F, Methawasin M, Adler M, Escobar YN, Nedrud J, Pappas CT, Harris SP, Granzier H.

J Mol Cell Cardiol. 2016 Aug;97:286-94. doi: 10.1016/j.yjmcc.2016.04.013. Epub 2016 Apr 30.

43.

Mutation-specific effects on thin filament length in thin filament myopathy.

Winter JM, Joureau B, Lee EJ, Kiss B, Yuen M, Gupta VA, Pappas CT, Gregorio CC, Stienen GJ, Edvardson S, Wallgren-Pettersson C, Lehtokari VL, Pelin K, Malfatti E, Romero NB, Engelen BG, Voermans NC, Donkervoort S, Bönnemann CG, Clarke NF, Beggs AH, Granzier H, Ottenheijm CA.

Ann Neurol. 2016 Jun;79(6):959-69. doi: 10.1002/ana.24654. Epub 2016 Apr 30.

44.

Renin overexpression leads to increased titin-based stiffness contributing to diastolic dysfunction in hypertensive mRen2 rats.

Kovács Á, Fülöp GÁ, Kovács A, Csípő T, Bódi B, Priksz D, Juhász B, Beke L, Hendrik Z, Méhes G, Granzier HL, Édes I, Fagyas M, Papp Z, Barta J, Tóth A.

Am J Physiol Heart Circ Physiol. 2016 Jun 1;310(11):H1671-82. doi: 10.1152/ajpheart.00842.2015. Epub 2016 Apr 8.

45.

Phosphorylating Titin's Cardiac N2B Element by ERK2 or CaMKIIδ Lowers the Single Molecule and Cardiac Muscle Force.

Perkin J, Slater R, Del Favero G, Lanzicher T, Hidalgo C, Anderson B, Smith JE 3rd, Sbaizero O, Labeit S, Granzier H.

Biophys J. 2015 Dec 15;109(12):2592-2601. doi: 10.1016/j.bpj.2015.11.002.

46.

Role of Titin Missense Variants in Dilated Cardiomyopathy.

Begay RL, Graw S, Sinagra G, Merlo M, Slavov D, Gowan K, Jones KL, Barbati G, Spezzacatene A, Brun F, Di Lenarda A, Smith JE, Granzier HL, Mestroni L, Taylor M; Familial Cardiomyopathy Registry.

J Am Heart Assoc. 2015 Nov 13;4(11). pii: e002645. doi: 10.1161/JAHA.115.002645.

47.

Reduced passive force in skeletal muscles lacking protein arginylation.

Leite FS, Minozzo FC, Kalganov A, Cornachione AS, Cheng YS, Leu NA, Han X, Saripalli C, Yates JR 3rd, Granzier H, Kashina AS, Rassier DE.

Am J Physiol Cell Physiol. 2016 Jan 15;310(2):C127-35. doi: 10.1152/ajpcell.00269.2015. Epub 2015 Oct 28.

48.

Knockout of Lmod2 results in shorter thin filaments followed by dilated cardiomyopathy and juvenile lethality.

Pappas CT, Mayfield RM, Henderson C, Jamilpour N, Cover C, Hernandez Z, Hutchinson KR, Chu M, Nam KH, Valdez JM, Wong PK, Granzier HL, Gregorio CC.

Proc Natl Acad Sci U S A. 2015 Nov 3;112(44):13573-8. doi: 10.1073/pnas.1508273112. Epub 2015 Oct 20.

49.

Myosin light chain phosphorylation to the rescue.

Granzier HL, de Tombe PP.

Proc Natl Acad Sci U S A. 2015 Jul 28;112(30):9148-9. doi: 10.1073/pnas.1511455112. Epub 2015 Jul 8. No abstract available.

50.

Nebulin deficiency in adult muscle causes sarcomere defects and muscle-type-dependent changes in trophicity: novel insights in nemaline myopathy.

Li F, Buck D, De Winter J, Kolb J, Meng H, Birch C, Slater R, Escobar YN, Smith JE 3rd, Yang L, Konhilas J, Lawlor MW, Ottenheijm C, Granzier HL.

Hum Mol Genet. 2015 Sep 15;24(18):5219-33. doi: 10.1093/hmg/ddv243. Epub 2015 Jun 29.

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