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Research
General Information
ZIRC
ZFIN ID: ZDB-PERS-970807-2
Hughes, Simon M.
Email: simon.hughes@kcl.ac.uk
URL: http://www.kcl.ac.uk/lsm/research/divisions/randall/research/sections/signalling/hughes/index.aspx
Affiliation: Simon M. Hughes Lab
Address: Randall Division of Cell and Molecular Biophysics King's College London 3rd Floor North New Hunt's House Guy's Campus London, SE1 1UL United Kingdom
Country: United Kingdom
Phone: 44-20-7848-6445
Fax: 44-20-7848-6435
Orcid ID: 0000-0001-8227-9225


BIOGRAPHY AND RESEARCH INTERESTS
OBJECTIVES:

To elucidate the interaction of genetics and environment in regulating phenotype of a specific tissue: striated muscle. To understand the formation, maintenance, growth and repair of fast and slow muscle fibres, their attachments and innervation, in their anatomical context. In a word: to study cell biology in vivo.

ORGANISMS:

We work on zebrafish (mainly), Xenopus, chick and mouse muscle development, growth and repair. We also study effects of environment such as training, exercise or aging.

METHODOLOGY:

Molecular developmental genetics and cell biology. Strong emphasis on development of in vivo imaging.

BIOGRAPHY:

Ph.D., 1980-1984, Cambridge University, Dr. Martin Brand - Visual transduction in retinal rod cells.

Postdoc, 1985-1987, University College London, Professor Martin Raff - Glial cell development in optic nerve.

Postdoc, 1987-1991, Stanford University, Professor Helen Blau - Mammalian muscle development.

Principle Investigator, 1992-2002, MRC Muscle and Cell Motility Unit and Developmental Biology Research Centre, King's College London.

Principle Investigator, 2002-date, MRC External Scientific Staff; Professor of Developmental Cell Biology, Randall Division for Cell and Molecular Biophysics, King's College London and member of MRC Centre for Developmental Neurobiology.


PUBLICATIONS
Ciano, M., Mantellato, G., Connolly, M., Paul-Clark, M., Willis-Owen, S., Moffatt, M.F., Cookson, W.O.C.M., Mitchell, J.A., Polkey, M.I., Hughes, S.M., Kemp, P.R., Natanek, S.A. (2019) EGF receptor (EGFR) inhibition promotes a slow-twitch oxidative, over a fast-twitch, muscle phenotype. Scientific Reports. 9:9218
Ganassi, M., Badodi, S., Ortuste Quiroga, H.P., Zammit, P.S., Hinits, Y., Hughes, S.M. (2018) Myogenin promotes myocyte fusion to balance fibre number and size. Nature communications. 9:4232
Roy, S.D., Williams, V.C., Pipalia, T.G., Li, K., Hammond, C.L., Knappe, S., Knight, R.D., Hughes, S.M. (2017) Myotome adaptability confers developmental robustness to somitic myogenesis in response to fibre number alteration. Developmental Biology. 431(2):321-335
Fin, L., Bergamin, G., Steiner, R.A., Hughes, S.M. (2017) The Cannabinoid Receptor Interacting Proteins 1 of zebrafish are not required for morphological development, viability or fertility. Scientific Reports. 7:4858
Pipalia, T.G., Koth, J., Roy, S.D., Hammond, C.L., Kawakami, K., Hughes, S.M. (2016) Cellular dynamics of regeneration reveals role of two distinct Pax7 stem cell populations in larval zebrafish muscle repair. Disease models & mechanisms. 9(6):671-84
Bajanca, F., Gonzalez-Perez, V., Gillespie, S.J., Beley, C., Garcia, L., Theveneau, E., Sear, R.P., Hughes, S.M. (2015) In vivo dynamics of skeletal muscle Dystrophin in zebrafish embryos revealed by improved FRAP analysis. eLIFE. 4
Attili, S., Hughes, S.M. (2014) Anaesthetic Tricaine Acts Preferentially on Neural Voltage-Gated Sodium Channels and Fails to Block Directly Evoked Muscle Contraction. PLoS One. 9:e103751
Ganassi, M., Badodi, S., Polacchini, A., Baruffaldi, F., Battini, R., Hughes, S.M., Hinits, Y., Molinari, S. (2014) Distinct functions of alternatively spliced isoforms encoded by zebrafish mef2ca and mef2cb. Biochimica et biophysica acta. Gene regulatory mechanisms. 1839(7):559-70
Kulaveerasingam, D., Hinits, Y., Hughes, S. (2014) Modelling cardiac hypertrophy/hyperplasia in the zebrafish. Cardiovascular research. 103 Suppl 1:S58
Yogev, O., Williams, V.C., Hinits, Y., and Hughes, S.M. (2013) eIF4EBP3L acts as a gatekeeper of TORC1 in activity-dependent muscle growth by specifically regulating Mef2ca translational initiation. PLoS Biology. 11(10):e1001679
Minchin, J.E., Williams, V.C., Hinits, Y., Low, S., Tandon, P., Fan, C.M., Rawls, J.F., and Hughes, S.M. (2013) Oesophageal and sternohyal muscle fibres are novel Pax3-dependent migratory somite derivatives essential for ingestion. Development (Cambridge, England). 140(14):2972-2984
Pascoal, S., Esteves de Lima, J., Leslie, J.D., Hughes, S.M., and Saúde, L. (2013) Notch signalling is required for the formation of structurally stable muscle fibres in zebrafish. PLoS One. 8(6):e68021
Hinits, Y., Pan, L., Walker, C., Dowd, J., Moens, C.B., and Hughes, S.M. (2012) Zebrafish Mef2ca and Mef2cb are essential for both first and second heart field cardiomyocyte differentiation. Developmental Biology. 369(2):199-210
Hinits, Y., Williams, V.C., Sweetman, D., Donn, T.M., Ma, T.P., Moens, C.B., and Hughes, S.M. (2011) Defective cranial skeletal development, larval lethality and haploinsufficiency in Myod mutant zebrafish. Developmental Biology. 358(1):102-12
Osborn, D.P., Li, K., Hinits, Y., and Hughes, S.M. (2011) Cdkn1c drives muscle differentiation through a positive feedback loop with Myod. Developmental Biology. 350(2):464-475
Hinits, Y., Osborn, D.P., and Hughes, S.M. (2009) Differential requirements for myogenic regulatory factors distinguish medial and lateral somitic, cranial and fin muscle fibre populations. Development (Cambridge, England). 136(3):403-414
Minchin, J.E., and Hughes, S.M. (2008) Sequential actions of Pax3 and Pax7 drive xanthophore development in zebrafish neural crest. Developmental Biology. 317(2):508-522
Diogo, R., Hinits, Y., and Hughes, S. (2008) Development of mandibular, hyoid and hypobranchial muscles in the zebrafish: homologies and evolution of these muscles within bony fishes and tetrapods. BMC Developmental Biology. 8:24
Böhm, S., Jin, H., Hughes, S.M., Roberts, R.G., and Hinits, Y. (2008) Dystrobrevin and dystrophin family gene expression in zebrafish. Gene expression patterns : GEP. 8(2):71-78
Sparrow, J., Hughes, S.M., and Segalat, L. (2008) Other model organisms for sarcomeric muscle diseases. Advances in experimental medicine and biology. 642:192-206
Mann, C.J., Osborn, D.P., and Hughes, S.M. (2007) Vestigial-like-2b (VITO-1b) and Tead-3a (Tef-5a) expression in zebrafish skeletal muscle, brain and notochord. Gene expression patterns : GEP. 7(8):827-836
Hinits, Y., Osborn, D.P., Carvajal, J.J., Rigby, P.W., and Hughes, S.M. (2007) Mrf4 (myf6) is dynamically expressed in differentiated zebrafish skeletal muscle. Gene expression patterns : GEP. 7(7):738-745
Hinits, Y., and Hughes, S.M. (2007) Mef2s are required for thick filament formation in nascent muscle fibres. Development (Cambridge, England). 134(13):2511-2519
Hammond, C.L., Hinits, Y., Osborn, D.P., Minchin, J.E., Tettamanti, G., and Hughes, S.M. (2007) Signals and myogenic regulatory factors restrict pax3 and pax7 expression to dermomyotome-like tissue in zebrafish. Developmental Biology. 302(2):504-521
Jin, H., Tan, S., Hermanowski, J., Bohem, S., Pacheco, S., McCaule, J.M., Greener, M.J., Hinits, Y., Hughes, S.M., Sharpe, P.T., Roberts, R.G., (2007) The dystrotelin, dystrophin and dystrobrevin superfamily: new paralogues and old isoforms. BMC Genomics. 8(1):19
Mann, C.J., Hinits, Y., and Hughes, S.M. (2006) Comparison of neurolin (ALCAM) and neurolin-like cell adhesion molecule (NLCAM) expression in zebrafish. Gene expression patterns : GEP. 6(8):952-963
Devoto, S.H., Stoiber, W., Hammond, C.L., Steinbacher, P., Haslett, J.R., Barresi, M.J., Patterson, S.E., Adiarte, E.G., and Hughes, S.M. (2006) Generality of vertebrate developmental patterns: evidence for a dermomyotome in fish. Evolution & development. 8(1):101-110
Groves, J.A., Hammond, C.L., and Hughes, S.M. (2005) Fgf8 drives myogenic progression of a novel lateral fast muscle fibre population in zebrafish. Development (Cambridge, England). 132(19):4211-4222
Grimaldi, A., Tettamanti, G., Martin, B.L., Gaffield, W., Pownall, M.E., and Hughes, S.M. (2004) Hedgehog regulation of superficial slow muscle fibres in Xenopus and the evolution of tetrapod trunk myogenesis. Development (Cambridge, England). 131(14):3249-3262
Hughes, S.M. (2004) Muscle differentiation: a gene for slow muscle?. Current biology : CB. 14(4):156-157
Hampson, R. and Hughes, S.M. (2003) Acridine mutagenesis of zebrafish (Danio rerio). Mutation research. 525(1-2):1-9
Coutelle, O., Blagden, C.S., Hampson, R., Halai, C., Rigby, P.W.J., and Hughes, S.M. (2001) Hedgehog signalling is required for maintenance of myf5 and myoD expression and timely terminal differentiation in zebrafish adaxial myogenesis. Developmental Biology. 236(1):136-150
Blagden, C.S. and Hughes, S.M. (1999) Extrinsic influences on limb muscle organisation. Cell and tissue research. 296(1):141-150
Hughes, S.M., Blagden, C.S., Li, X., and Grimaldi, A. (1998) The role of hedgehog proteins in vertebrate slow and fast skeletal muscle patterning. Acta physiologica Scandinavica. 163(3):S7-10
Blagden, C.S., P.D. Currie, P.W. Ingham, and S.M. Hughes (1997) Notochord induction of zebrafish slow muscle mediated by Sonic hedgehog. Genes & Development. 11(17):2163-2175

NON-ZEBRAFISH PUBLICATIONS
*Anderson, C., *Williams, V.C., Moyon, B., Daubas, P., Tajbakhsh, S., Buckingham, M.E., Shiroishi, T., Hughes, S.M. and A-G. Borycki (2012) Sonic hedgehog acts cell-autonomously on muscle precursor cells to generate limb muscle diversity. Genes Dev. 26:2103-17. PMID: 22987640
Madhala-Levy, D., Williams, V.C., Hughes, S.M., Reshef, R. and O. Halevy (2012) Cooperation between Shh and IGF-I in Promoting Myogenic Proliferation and Differentiation via the MAPK/ERK and PI3K/Akt Pathways Requires Smo Activity. J. Cell. Physiol. 227:1455-64 PMID: 21618536
• *Henriquez, J-P.. *Webb, A., Bence, M., Bildsoe, H., Sahores, M., Hughes, S.M. and P.C. Salinas (2008) Wnt signalling promotes AChR aggregation at the neuromuscular synapse in collaboration with Agrin. Proc. Natl. Acad. Sci. USA. 105: 18812-18817. PMID: 19020093
• Maggs, A.M., Huxley, C. and S.M. Hughes (2008) Nerve-dependent changes in skeletal muscle myosin heavy chain after experimental denervation, cross-reinnervation and in a demyelinating mouse model of Charcot-Marie-Tooth disease type 1A. Muscle Nerve. 38: 1572–1584, PMID: 19016545
• *Chargé, S.B., *Brack, A.S.,Bayol, S.A. and S.M. Hughes (2008) MyoD- and nerve-dependent maintenance of MyoD expression in mature muscle fibres acts through the DRR/PRR element. BMC Devel. Biol. 8: 5. PMID: 18215268
• Lange, S., Xiang, F., Yakovenko, A., Vihola, A., Rostkova, E., Hackman, P., Kristensen, J., Brandmeier, B., Franzen, G., Hedberg, B. et al. (2005). The kinase domain of titin controls muscle gene expression and protein turnover. Science 308:1599-1603.
• Brack, A.S., Bildsoe, H. and S.M. Hughes. (2005) Evidence that satellite cell decrement contributes to preferential decline in nuclear number from large fibres during murine age-related muscle atrophy J. Cell Sci. 118:4813-4821.
• Bruusgaard, J.C., Brack, A.S., Hughes, S.M. and K. Gundersen (2005) Muscle hypertrophy induced by the Ski protein: cyto-architecture and ultrastructure. Acta Physiol. Scand. 185:141-149.
• *Schuierer, M.M., *Mann, C.J., Bildsoe, H., Huxley, C. and S.M. Hughes (2005) Analyses of the differentiation potential of satellite cells from myoD-/-, mdx, and PMP22 C22 mice. BMC Musculoskeletal Disord. 6:15.
• *Li, X., *Blagden, C.S., *Bildsoe, H., Bonnin, M-A., Duprez, D. and S.M. Hughes (2004) Hedgehog can drive terminal differentiation of amniote slow skeletal muscle. BMC Dev. Biol. 4: 9.
• Grimaldi, A., Tettamanti, G., Martin, B.,L. Gaffield, W., Pownall, M.E. and S.M. Hughes (2004) Hedgehog regulation of superficial slow muscle fibres in Xenopus and the evolution of tetrapod trunk myogenesis. Development. 131: 3249-3262.
• Jazwinska, A., Elher, E. and S.M. Hughes (2003) Intermediate filament-co-localized myosin heavy chain-like molecules define distinct cellular domains in hair follicles and epidermis (2003) BMC Cell Biology 4(1): 10.
• Krylova, O., Herreros, J. Cleverley, K., Ehler, E., Hughes, S.M. and P.C. Salinas. (2002) Wnt-3, expressed by motoneurons, regulates terminal arborization of neurotrophin-3-responsive spinal sensory neurons. Neuron 35: 1043-1056.
• Chargé, S.B., Brack, A.S. and S.M. Hughes (2002) Aging-related satellite cell differentiation defect occurs prematurely after Ski-induced skeletal muscle hypertrophy. Am. J. Physiol. 283: C1228-C1241.
• Lluis, F., Roma, J., Suelves, M., Parra, M., Aniorte, G., Gallardo, E., Illa, I., Rodriguez, L., Hughes, S.M., Carmeliet, P., Roig, M. and P. Munoz-Canoves (2001) Urokinase-dependent plasminogen activation is required for efficient skeletal muscle regeneration in vivo. Blood 97: (6) 1703-1711.
• Maggs, A.M., Taylor-Harris, P., Peckham, M. and S.M. Hughes (2000) Evidence for differential post-translational modifications of slow myosin heavy chain during murine skeletal muscle development. J. Muscle Res. Cell Motil. 21: (2) 101-113.
• Hook, P., Li, X., Sleep, J., Hughes, S.M. and L. Larsson (1999) Motility of slow myosin extracted from single muscle cells in young and old rat soleus muscle. J. Physiol. 520: 463-471.
• Hughes, S.M., Chi, M.M-Y., Lowry, O.H. and K. Gundersen (1999) Myogenin induces a shift in enzyme activity from glycolytic to oxidative metabolism in muscles of transgenic mice. J. Cell. Biol. 145(3):633-642.
• Robson, LG. and S.M. Hughes (1999) Local signals in the chick limb bud can override myoblast lineage commitment: induction of slow myosin hevay chain in fast myoblasts. Mech. Dev. 85: 59-71.
• Hughes, S.M. (1999) Fetal myoblast clones contribute to both fast and slow fibres in developing rat muscle. Int. J. Dev. Biol. 43:149-155.
• Hughes, S.M. and P.C. Salinas (1999) Control of muscle fibre and motoneuron diversification. Curr. Opin. Neurobiol. 9, 54-64.
• Blagden, C.S. and S.M. Hughes (1999) Extrinsic influences on limb muscle organisation. Cell Tissue Res. 296, 141-150.
• Hughes, S.M. (1998) Muscle development: electrical control of gene expression. Current Biology. 8, R892-R894.
• Hughes, S.M., C.S. Blagden, X. Li and A. Grimaldi. (1998) The role of Hedgehog proteins in vertebrate slow and fast skeletal muscle patterning. Acta Physiol. Scand. 163, S7-S10.
• Blake, J., Salinas, P.C. and Hughes, S.M. (1997) nßgeo, a combined selection and reporter gene for retroviral and transgenic studies. Biotechniques, 23: 690-695.
• Hughes, S.M., Koishi, K., Rudnicki, M. and Maggs, A.M. (1997) MyoD protein is differentially accumulation in fast and slow skeletal muscle and required for normal fibre type balance in rodents. Mech. Dev. 61: 151-163.
• Li, X., S.M. Hughes, G. Salviati, A. Teresi and L. Larsson (1996) Thyroid hormone effects on contractility and myosin composition of soleus muscle and single fibres from young and old rats. J. Physiol. 494: 555-567.
• Robson, L.G. and S.M. Hughes (1996) The distal limb environment regulates MyoD accumulation and muscle differentiation in mouse-chick chimaeric limbs. Development 122: 3389-3910.