Imperial College London

ProfessorSteveMarston

Faculty of MedicineNational Heart & Lung Institute

(Non-Clinical) Professor in Cardiovascular Biochemistry
 
 
 
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Contact

 

+44 (0)20 7594 2732s.marston Website

 
 
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Location

 

433ICTEM buildingHammersmith Campus

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Summary

 

Publications

Publication Type
Year
to

188 results found

Copeland O, Nowak KJ, Laing NG, Ravenscroft G, Messer AE, Bayliss CR, Marston SBet al., 2010, Investigation of changes in skeletal muscle alpha-actin expression in normal and pathological human and mouse hearts, JOURNAL OF MUSCLE RESEARCH AND CELL MOTILITY, Vol: 31, Pages: 207-214, ISSN: 0142-4319

Journal article

Song W, Dyer E, Stuckey D, Leung M-C, Memo M, Mansfield C, Ferenczi M, Liu K, Redwood C, Nowak K, Harding S, Clarke K, Wells D, Marston Set al., 2010, Investigation of a transgenic mouse model of familial dilated cardiomyopathy, JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY, Vol: 49, Pages: 380-389, ISSN: 0022-2828

Journal article

Messer AE, Gallon CE, McKenna WJ, Dos Remedios CG, Marston SBet al., 2009, The use of phosphate-affinity SDS-PAGE to measure the cardiac troponin I phosphorylation site distribution in human heart muscle, PROTEOMICS CLINICAL APPLICATIONS, Vol: 3, Pages: 1371-1382, ISSN: 1862-8346

Journal article

Dyer EC, Jacques AM, Hoskins AC, Ward DG, Gallon CE, Messer AE, Kaski JP, Burch M, Kentish JC, Marston SBet al., 2009, Functional Analysis of a Unique Troponin C Mutation, GLY159ASP, that Causes Familial Dilated Cardiomyopathy, Studied in Explanted Heart Muscle, CIRCULATION-HEART FAILURE, Vol: 2, Pages: 456-U101, ISSN: 1941-3289

Journal article

Marston S, Copeland O, Jacques A, Livesey K, Tsang V, McKenna WJ, Jalilzadeh S, Carballo S, Redwood C, Watkins Het al., 2009, Evidence From Human Myectomy Samples That MYBPC3 Mutations Cause Hypertrophic Cardiomyopathy Through Haploinsufficiency, CIRCULATION RESEARCH, Vol: 105, Pages: 219-U39, ISSN: 0009-7330

Journal article

Marston SB, Walker JW, 2009, Back to the future: new techniques show that forgotten phosphorylation sites are present in contractile proteins of the heart whilst intensively studied sites appear to be absent, JOURNAL OF MUSCLE RESEARCH AND CELL MOTILITY, Vol: 30, Pages: 93-95, ISSN: 0142-4319

Journal article

Feng J-J, Ushakov DS, Ferenczi MA, Laing NG, Nowak KJ, Marston SBet al., 2009, Direct visualisation and kinetic analysis of normal and nemaline myopathy actin polymerisation using total internal reflection microscopy, JOURNAL OF MUSCLE RESEARCH AND CELL MOTILITY, Vol: 30, Pages: 85-92, ISSN: 0142-4319

Journal article

Song W, Stuckey DJ, Dyer E, Wells D, Harding SE, Carr CA, Clarke K, Marston SBet al., 2009, Mouse HCM Model Expressing E99K ACTC Mutation Reproduces Phenotypes As Found In Human Patients, Publisher: CELL PRESS, Pages: 499A-500A, ISSN: 0006-3495

Conference paper

Messer AE, Gallon CE, Marston SB, 2009, Analysis of Cardiac Myofibrillar Troponin I Phosphorylation in Normal and Failing Human Hearts Using Phos-Tags, Publisher: CELL PRESS, Pages: 501A-501A, ISSN: 0006-3495

Conference paper

Marston S, Copeland O, Jacques A, McKenna W, Tsang V, Livesey K, Carballo S, Redwood C, Watkins Het al., 2009, Direct Evidence In Man For Haploinsufficiency As The Mechanism Of Action Of Myosin-binding Protein C Mutations That Cause Hypertrophic Cardiomyopathy, Publisher: CELL PRESS, Pages: 371A-371A, ISSN: 0006-3495

Conference paper

Copeland O, Ravenscroft G, Nowak K, Laing N, Marston Set al., 2009, Quantitative Assay of Skeletal Muscle alpha-actin Expression In Normal and Pathological Human and Mouse Hearts, Publisher: CELL PRESS, Pages: 499A-499A, ISSN: 0006-3495

Conference paper

Feng J-J, Marston S, 2009, Genotype-phenotype correlations in ACTA1 mutations that cause congenital myopathies, NEUROMUSCULAR DISORDERS, Vol: 19, Pages: 6-16, ISSN: 0960-8966

Journal article

Hamdani N, de Waard M, Messer AE, Boontje NM, Kooij V, van Dijk S, Versteilen A, Lamberts R, Merkus D, dos Remedios C, Duncker DJ, Borbely A, Papp Z, Paulus W, Stienen GJM, Marston SB, van der Velden Jet al., 2008, Myofilament dysfunction in cardiac disease from mice to men, JOURNAL OF MUSCLE RESEARCH AND CELL MOTILITY, Vol: 29, Pages: 189-201, ISSN: 0142-4319

Journal article

Jacques A, Hoskins AC, Kentish JC, Marston SBet al., 2008, From genotype to phenotype: a longitudinal study of a patient with hypertrophic cardiomyopathy due to a mutation in the MYBPC3 gene, JOURNAL OF MUSCLE RESEARCH AND CELL MOTILITY, Vol: 29, Pages: 239-246, ISSN: 0142-4319

Journal article

Marston SB, de Tombe PP, 2008, Troponin phosphorylation and myofilament Ca2+ -sensitivity in heart failure: Increased or decreased?, JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY, Vol: 45, Pages: 603-607, ISSN: 0022-2828

Journal article

Jacques AM, Briceno N, Messer AE, Gallon CE, Jalilzadeh S, Garcia E, Kikonda-Kanda G, Goddard J, Harding SE, Watkins H, Esteban MT, Tsang VT, McKenna WJ, Marston SBet al., 2008, The molecular phenotype of human cardiac myosin associated with hypertrophic obstructive cardiomyopathy, CARDIOVASCULAR RESEARCH, Vol: 79, Pages: 481-491, ISSN: 0008-6363

Journal article

Marston S, 2008, How does genotype define phenotype? Microphysiology of a tropomyosin mutation in situ shows the limitations of reductionism, JOURNAL OF PHYSIOLOGY-LONDON, Vol: 586, Pages: 2821-2821, ISSN: 0022-3751

Journal article

Ansari S, Alahyan M, Marston SB, EL-Mezgueldi Met al., 2008, Role of caldesmon in the Ca2+ regulation of smooth muscle thin filaments, JOURNAL OF BIOLOGICAL CHEMISTRY, Vol: 283, Pages: 47-56

Journal article

Matsson H, Eason J, Bookwalter CS, Klar J, Gustavsson P, Sunnegardh J, Enell H, Jonzon A, Vikkula M, Gutierrez I, Granados-Riveron J, Pope M, Bu'Lock F, Cox J, Robinson TE, Song F, Brook DJ, Marston S, Trybus KM, Dahl Net al., 2008, Alpha-cardiac actin mutations produce atrial septal defects, HUMAN MOLECULAR GENETICS, Vol: 17, Pages: 256-265, ISSN: 0964-6906

Journal article

Marston S, El-Mezgueldi M, 2008, Role of Tropomyosin in the Regulation of Contraction in Smooth Muscle, TROPOMYOSIN, Vol: 644, Pages: 110-123, ISSN: 0065-2598

Journal article

Jacques A, Copeland O, Messer A, Gallon C, King C, McKenna W, Tsang V, Marston Set al., 2008, Myosin binding protein C phosphorylation in normal, hypertrophic and failing human heart muscle., J Mol Cell Cardiol, Vol: 45, Pages: 209-216

Phosphorylation of myosin binding protein C (MyBP-C) was investigated in intraventricular septum samples taken from patients with hypertrophic cardiomyopathy undergoing surgical septal myectomy. These samples were compared with donor heart muscle, as a well-characterised control tissue, and with end-stage failing heart muscle. MyBP-C was partly purified from myofibrils using a modification of the phosphate-EDTA extraction of Hartzell and Glass. MyBP-C was separated by SDS-PAGE and stained for phosphoproteins using Pro-Q Diamond followed by total protein staining using Coomassie Blue. Relative phosphorylation level was determined from the ratio of Pro-Q Diamond to Coomassie Blue staining of MyBP-C bands as measured by densitometry. We compared 9 myectomy samples and 9 failing heart samples with 9 donor samples. MyBP-C phosphorylation in pathological muscle was lower than in donor (myectomy 40+/-2% of donor, P<0.0001; failing 45+/-3% of donor, P<0.0001). 6 myectomy samples were identified with MYBPC3 mutations, one with MYH7 mutation and two remained unknown, but there was no correlation between MYBPC3 mutation and MyBP-C phosphorylation level. In order to determine the number of phosphorylated sites in human cardiac MyBP-C samples, we phosphorylated the recombinant MyBP-C fragment, C0-C2 (1-453) with PKA using (gamma32)P-ATP up to 3.5 mol Pi/mol C0-C2. This measurement of phosphorylation was used to calibrate measurements of phosphorylation in SDS-PAGE using Pro-Q Diamond stain. The level of phosphorylation in donor heart MyBP-C was calculated to be 4.6+/-0.6 mol Pi/mol and 2.0+/-0.3 mol Pi/mol in myectomy samples. We conclude that MyBP-C is a highly phosphorylated protein in vivo and that diminished MyBP-C phosphorylation is a feature of both end-stage heart failure and hypertrophic cardiomyopathy.

Journal article

Kasakov AS, Bukach OV, Seit-Nebi AS, Marston SB, Gusev NBet al., 2007, Effect of mutations in the beta 5-beta 7 loop on the structure and properties of human small heat shock protein HSP22 (HspB8, H11), FEBS JOURNAL, Vol: 274, Pages: 5628-5642, ISSN: 1742-464X

Journal article

Clarke NF, Ilkovski B, Cooper S, Valova VA, Robinson PJ, Nonaka I, Feng J-J, Marston S, North Ket al., 2007, The pathogenesis of ACTA1-related congenital fiber type disproportion, ANNALS OF NEUROLOGY, Vol: 61, Pages: 552-561, ISSN: 0364-5134

Journal article

Mirza M, Robinson P, Kremneva E, Copeland O, Nikolaeva O, Watkins H, Levitsky D, Redwood C, EL-Mezgueldi M, Marston Set al., 2007, The effect of mutations in alpha-tropomyosin (E40K and E54K) that cause familial dilated cardiomyopathy on the regulatory mechanism of cardiac muscle thin filaments, JOURNAL OF BIOLOGICAL CHEMISTRY, Vol: 282, Pages: 13487-13497, ISSN: 0021-9258

Journal article

Chernik IS, Seit-Nebi AS, Marston SB, Gusev NBet al., 2007, Small heat shock protein Hsp20 (HspB6) as a partner of 14-3-3 gamma, MOLECULAR AND CELLULAR BIOCHEMISTRY, Vol: 295, Pages: 9-17, ISSN: 0300-8177

Journal article

Messer AE, Marston SB, Jacques AM, 2007, Troponin phosphorylation and regulatory function in human heart muscle: dephosphorylation of Ser23/24 on troponin I could account for the contractile defect in end-stage heart failure, Journal of Molecular and Cellular Cardiology, Vol: 42, Pages: 247-259

Journal article

D'Amico A, Graziano C, Pacileo G, Petrini S, Nowak KJ, Boldrini R, Jacques A, Feng J-J, Porfirio B, Sewry CA, Santorelli FM, Limongelli G, Bertini E, Laing N, Marston SBet al., 2006, Fatal hypertrophic cardiomyopathy and nemaline myopathy associated with ACTA1 K336E mutation, NEUROMUSCULAR DISORDERS, Vol: 16, Pages: 548-552, ISSN: 0960-8966

Journal article

Kim MV, Kasakov AS, Seit-Nebi AS, Marston SB, Gusev NBet al., 2006, Structure and properties of K141E mutant of small heat shock protein HSP22 (HspB8, H11) that is expressed in human neuromuscular disorders, ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS, Vol: 454, Pages: 32-41, ISSN: 0003-9861

Journal article

Alahyan M, Webb MR, Marston SB, El-Mezgueldi Met al., 2006, The mechanism of smooth muscle caldesmon-tropomyosin inhibition of the elementary steps of the actomyosin ATPase, JOURNAL OF BIOLOGICAL CHEMISTRY, Vol: 281, Pages: 19433-19448

Journal article

Mirza M, Marston S, Willott R, Ashley C, Mogensen J, McKenna W, Robinson P, Redwood C, Watkins Het al., 2005, Dilated cardiomyopathy mutations in three thin filament regulatory proteins result in a common functional phenotype, JOURNAL OF BIOLOGICAL CHEMISTRY, Vol: 280, Pages: 28498-28506

Journal article

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