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Professor Paul Chapple

Paul

Professor of Molecular Cell Biology and Deputy Dean for Postgraduate Research

Centre: Endocrinology

Email: j.p.chapple@qmul.ac.uk
Telephone: +44(0) 20 7882 6242

Profile

ORCID iD: 0000-0003-1876-1505

Professor Paul Chapple was awarded a PhD by University College London in 1997, this investigated the physiological and evolutionary significance of Hsp70 chaperones in a marine organism. He then worked for eight years as a Postdoctoral Researcher in the laboratory of Professor Michael Cheetham at the UCL Institute of Ophthalmology, where he researched the cell biology of molecular chaperone proteins involved in neurodegeneration and blindness. In 2004 he moved to the KCL Institute of Psychiatry to work with Dr Jean-Marc Gallo on the Alzheimer’s protein Tau. Professor Chapple became a Lecturer at the William Harvey Research Institute (WHRI) in 2006, was promoted to Reader in 2010 and Professor in 2014. He has served as a reviewer and committee member for several grant-giving bodies, including BBSRC and the Society for Endocrinology. Current academic roles include that he is the School of Medicine and Dentistry Deputy Dean for Postgraduate Research and the Co-Centre Lead for the Centre for Endocrinology.

Research

Group members

  • PhD students: Mohammed Dushti
  • Clinical research fellows: Dr Jalil-Ahmad Sharif, Dr Eugenie Lim
  • Postdoctoral researchers: Dr Grace Salsbury, Dr Laura Perna 

Summary

Cell stress and molecular chaperones in human disease
My research concentrates on understanding the role of cell stress and molecular chaperones in health and disease, with a focus on neuronal and endocrine systems.

It is critical for cell survival that protein homeostasis (proteostasis) is maintained. Molecular chaperones are essential modulators of proteostasis networks, regulating aspects of protein quality control, such as folding and degradation. One of my particular interest is understanding how chaperone systems are specialised in different cell types and organelles.

Ongoing projects include:

J proteins in neurodegenerative disorders
J-domain cochaperones are important for neuronal health. This includes the protein sacsin that is mutated in an early onset ataxia. Loss of sacsin disrupts intermediate filament organisation and mitochondrial function. We hypothesise that sacsin functions as a chaperone for specific clients and are currently identifying these proteins to understand disease mechanism.

Cell biology of Pheochromocytoma
Pheochromocytoma are rare, catecholamine-secreting tumors that may precipitate life-threatening hypertension. We are investigating cellular mechanisms of tumorigenesis in pheochromocytoma with a current focus on hypoxia induced signaling pathways.

Chaperones and environmental modulation of primary cilia function
Primary cilia play a role in the coordination of cellular signaling pathways, with dysfunction associated with genetic syndromes and other disease. We are investigating the requirement for chaperones in cilia function and mechanisms that regulate cilia response to the cellular stress.

G protein-coupled receptor trafficking
GPCRs respond to a wide range of extracellular stimuli and are important drug targets. We are elucidating the chaperones involved in GPCR quality control and identifying strategies to rescue cell surface expression of intracellular retained receptor mutants. This research is relevant to pathologies including monogenic obesity and retinal degeneration.

Publications

Full list of publications

  • Perna L, Salsbury G, Dushti M et al. (publicationYear). Altered Cellular Metabolism Is a Consequence of Loss of the Ataxia-Linked Protein Sacsin. nameOfConference


    QMRO: qmroHref
  • Mcleod JC, Lim C, Stokes T et al. (publicationYear). Network-based modelling reveals cell-type enriched patterns of non-coding RNA regulation during human skeletal muscle remodelling. nameOfConference


    QMRO: qmroHref
  • Ozkan-Nikitaras T, Grzesik DJ, Romano LEL et al. (publicationYear). N-SREBP2 Provides a Mechanism for Dynamic Control of Cellular Cholesterol Homeostasis. nameOfConference


  • Marszalek J, De Los Rios P, Cyr D et al. (2024). J-domain proteins: From molecular mechanisms to diseases. nameOfConference


    QMRO: qmroHref
  • Perna L, Castelli M, Frasnetti E et al. (publicationYear). AlphaFold predicted structure of the Hsp90-like domains of the neurodegeneration linked protein sacsin reveals key residues for ATPase activity. nameOfConference


  • Prodromou C, Aran-Guiu X, Oberoi J et al. (2023). HSP70-HSP90 Chaperone Networking in Protein-Misfolding Disease. nameOfConference


    QMRO: qmroHref
  • Romano LEL, Aw WY, Hixson KM et al. (2022). Multi-omic profiling reveals the ataxia protein sacsin is required for integrin trafficking and synaptic organization. nameOfConference


  • Tufton N, Hearnden RJ, Berney DM et al. (2022). The immune cell infiltrate in the tumour microenvironment of phaeochromocytomas and paragangliomas. nameOfConference


    QMRO: qmroHref
  • Sladen PE, Jovanovic K, Guarascio R et al. (2022). Modelling autosomal dominant optic atrophy associated with OPA1 variants in iPSC-derived retinal ganglion cells. nameOfConference


  • Meireles CG, Lourenço de Lima C, Martins de Paula Oliveira M et al. (2022). Antiproliferative effects of metformin in cellular models of pheochromocytoma. nameOfConference


    QMRO: qmroHref
  • Sladen PE, Perdigão PRL, Salsbury G et al. (2021). CRISPR-Cas9 correction of OPA1 c.1334G>A: p.R445H restores mitochondrial homeostasis in dominant optic atrophy patient-derived iPSCs. nameOfConference


  • Callender LA, Schroth J, Carroll EC et al. (2021). GATA3 induces mitochondrial biogenesis in primary human CD4+ T cells during DNA damage. nameOfConference


  • Thompson C, Mcfie M, Chapple J et al. (2021). Polycystin-2 is required for chondrocyte mechanotransduction and traffics to the primary cilium in response to mechanical stimulation. nameOfConference


  • Desai R, East DA, Hardy L et al. (2020). Mitochondria form contact sites with the nucleus to couple prosurvival retrograde response. nameOfConference


    QMRO: qmroHref
  • Athanasiou D, Bevilacqua D, Aguila M et al. (2020). Corrigendum to: The co-chaperone and reductase ERdj5 facilitates rod opsin biogenesis and quality control. nameOfConference


    QMRO: qmroHref
  • Fu S, Thompson CL, Ali A et al. (2019). Mechanical loading inhibits cartilage inflammatory signalling via an HDAC6 and IFT-dependent mechanism regulating primary cilia elongation. nameOfConference


  • CHAPPLE JP, O'TOOLE S, WATSON D et al. (2019). Oncometabolite induced primary cilia loss in pheochromocytoma. nameOfConference


  • Costa ARD, Qarin S, Bradshaw T et al. (publicationYear). Can novel stem cell models help unpick the pathogenesis of the Triple A syndrome?. nameOfConference


    QMRO: qmroHref
  • Gentil BJ, Lai G-T, Menade M et al. (2019). Sacsin, mutated in the ataxia ARSACS, regulates intermediate filament assembly and dynamics. nameOfConference


    QMRO: qmroHref
  • Da Costa AR, Qarin S, Bradshaw TY et al. (2018). A Novel Stem Cell Model for the Triple a Syndrome. 57th Annual Meeting of the European Society for Paediatric Endocrinology (ESPE)


  • Parkinson MH, Bartmann AP, Clayton LMS et al. (2018). Optical coherence tomography in autosomal recessive spastic ataxia of Charlevoix-Saguenay. nameOfConference


  • Da CAR, Meimaridou E, Prasad R et al. (publicationYear). Novel evidence implies that ALADIN, the triple A syndrome gene product is involved in mitochondrial physiology. nameOfConference


    QMRO: qmroHref
  • Thompson CL, Plant JC, Wann AK et al. (2017). Chondrocyte expansion is associated with loss of primary cilia and disrupted hedgehog signalling.. nameOfConference


  • CHAPPLE JP (publicationYear). Altered organisation of the intermediate filament cytoskeleton and relocalisation of proteostasis modulators in cells lacking the ataxia protein sacsin. nameOfConference


  • Zhang J, Dalbay M, Luo X et al. (2017). Topography of calcium phosphate ceramics regulates primary cilia length and TGF receptor recruitment associated with osteogenesis. nameOfConference


  • Schwarz N, Lane A, Jovanovic K et al. (2017). Arl3 and RP2 regulate the trafficking of ciliary tip kinesins.. nameOfConference


  • Schwarz N, Lane A, Jovanovic K et al. (2017). Arl3 and RP2 regulate the trafficking of ciliary tip kinesins. (CORRIGENDUM). nameOfConference


  • Thompson CL, Chapple JP, Beales PL et al. (2017). Polycystins are required for primary cilia-mediated mechanotransduction in chondrocytes. nameOfConference

    DOI: doi

  • O'Toole SM, Chapple JP (2016). Primary cilia: a link between hormone signalling and endocrine-related cancers?. nameOfConference


    QMRO: qmroHref
  • CHAPPLE JP, Bradshaw TY, Romano LEL et al. (2016). A reduction in Drp1 mediated fission compromises mitochondrial health in autosomal recessive spastic ataxia of Charlevoix Saguenay. nameOfConference


  • O'Toole S, Srirangalingam U, Drake W et al. (publicationYear). The role of primary cilia in the molecular pathogenesis of phaeochromocytoma. nameOfConference


    QMRO: qmroHref
  • Kelly T-AN, Thompson CL, Tan E et al. (2016). Chondrocyte dedifferentiation down regulates mechano-responsiveness and hedgehog signalling associated with changes in primary cilia structure. World Congress of the Osteoarthritis-Research-Society-International (OARSI) on Osteoarthritis


    QMRO: qmroHref
  • Thompson CL, Patel R, Kelly T-AN et al. (2015). Hedgehog signalling does not stimulate cartilage catabolism and is inhibited by Interleukin-1β. nameOfConference


  • Dalbay MT, Thorpe SD, Connelly JT et al. (2015). Adipogenic Differentiation of hMSCs is Mediated by Recruitment of IGF‐1r Onto the Primary Cilium Associated With Cilia Elongation. nameOfConference


  • Blumkin L, Bradshaw T, Michelson M et al. (2015). Molecular and functional studies of retinal degeneration as a clinical presentation of SACS-related disorder. nameOfConference


    QMRO: qmroHref
  • Thompson CL, Wann AKT, Chapple JP et al. (2015). Competitive interactions between hedgehog and cytokine signalling: crosstalk at the chondrocyte primary cilium?. nameOfConference

    DOI: doi

  • Athanasiou D, Bevilacqua D, Aguila M et al. (2014). The co-chaperone and reductase ERdj5 facilitates rod opsin biogenesis and quality control.. nameOfConference


  • Duncan EJ, Cheetham ME, Chapple JP et al. (2015). The Role of HSP70 and Its Co-chaperones in Protein Misfolding, Aggregation and Disease. nameOfConference


    QMRO: qmroHref
  • Wann AKT, Chapple JP, Knight MM (2014). The primary cilium influences interleukin-1β-induced NFκB signalling by regulating IKK activity.. nameOfConference


  • Thompson CL, Chapple JP, Knight MM (2014). Primary cilia disassembly down-regulates mechanosensitive hedgehog signalling: a feedback mechanism controlling ADAMTS-5 expression in chondrocytes.. nameOfConference


  • Thompson CL, Chapple JP, Knight MM (2014). Primary cilia disassembly down regulates mechanosensitive hedgehog signalling: a feedback mechanism controlling ADAMTS-5 expression in chondrocytes. nameOfConference

    DOI: doi

  • Wann AKT, Thompson CL, Chapple JP et al. (2014). The chondrocyte cilium; a locality for HIF regulation in inflammatory signalling. nameOfConference

    DOI: doi

    QMRO: qmroHref
  • Wann AKT, Chapple JP, Knight MM (2014). The chondrocyte primary cilium is required for IL-1 induced NF-κβ signalling. nameOfConference

    DOI: doi

    QMRO: qmroHref
  • Wann AK, Thompson CL, Chapple JP et al. (2013). Interleukin-1β sequesters hypoxia inducible factor 2α to the primary cilium.. nameOfConference


  • Meimaridou E, Hughes CR, Kowalczyk J et al. (2013). Familial glucocorticoid deficiency: New genes and mechanisms. nameOfConference


    QMRO: qmroHref
  • Meimaridou E, Hughes CR, Kowalczyk J et al. (2013). Familial glucocorticoid deficiency: New genes and mechanisms.. nameOfConference


    QMRO: qmroHref
  • Prodromou NV, Thompson CL, Osborn DPS et al. (2012). Heat shock induces rapid resorption of primary cilia. nameOfConference


    QMRO: qmroHref
  • Chapple JP, Parfitt DA, Campbell DC (2012). Hsp70 Chaperone Systems in Vesicular Trafficking. nameOfConference


    QMRO: qmroHref
  • Thompson C, Prodromou N, Osborn D et al. (2012). Heat-shock induces rapid resorption of primary cilia. nameOfConference


    QMRO: qmroHref
  • Thompson C, Chapple J, Knight M (2012). Mechanical strain disrupts primary cilia structure and modulates hedgehog signalling in adult chondrocytes. nameOfConference


    QMRO: qmroHref
  • Prodromou NV, Thompson CL, Osborn DPS et al. (2012). Heat shock induces rapid resorption of primary cilia.. nameOfConference


    QMRO: qmroHref
  • Athanasiou D, Kosmaoglou M, Kanuga N et al. (2012). BiP prevents rod opsin aggregation.. nameOfConference


    QMRO: qmroHref
  • Chahal HS, Trivellin G, Leontiou CA et al. (2012). Somatostatin analogs modulate AIP in somatotroph adenomas: the role of the ZAC1 pathway.. nameOfConference


    QMRO: qmroHref
  • Meimaridou E, Kowalczyk J, Guasti L et al. (2012). Mutations in NNT encoding nicotinamide nucleotide transhydrogenase cause familial glucocorticoid deficiency.. nameOfConference


  • Giunti P, Nethisinghe S, Clayton LM et al. (2012). 002 Detecting retinal changes in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) using optical coherence tomography (OCT). nameOfConference


    QMRO: qmroHref
  • Thompson CL, Prodromou NV, Osborn DP et al. (2012). Heat-shock induces rapid resorption of primary cilia. nameOfConference

    DOI: doi

    QMRO: qmroHref
  • Thompson CL, Chapple JP, Knight MM (2012). MECHANICAL STRAIN STIMULATES HEDGEHOG SIGNALLING IN ADULT ARTICULAR CHONDROCYTES AND REDUCES PRIMARY CILIA LENGTH. nameOfConference


    QMRO: qmroHref
  • Thompson CL, Chapple JP, Knight MM (2012). Mechanical strain reduces primary cilia length and stimulates hedgehog signalling in adult chondrocytes. nameOfConference

    DOI: doi

    QMRO: qmroHref
  • Girard M, Lariviere R, Parfitt DA et al. (2012). Mitochondrial dysfunction and Purkinje cell loss in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS). nameOfConference


    QMRO: qmroHref
  • Parkinson MH, Nethisinghe S, Clayton L et al. (2012). Retinal changes in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) and other genetic ataxias. nameOfConference

    DOI: doi

    QMRO: qmroHref
  • Meimaridou E, Gooljar SB, Ramnarace N et al. (2011). The cytosolic chaperone Hsc70 promotes traffic to the cell surface of intracellular retained melanocortin-4 receptor mutants.. nameOfConference


    QMRO: qmroHref
  • Nethisinghe S, Clayton L, Vermeer S et al. (2011). Retinal Imaging in Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay. nameOfConference


    QMRO: qmroHref
  • Igreja S, Chahal HS, King P et al. (2010). Characterization of aryl hydrocarbon receptor interacting protein (AIP) mutations in familial isolated pituitary adenoma families.. nameOfConference


  • Chahal HS, Chapple JP, Frohman LA et al. (2010). Clinical, genetic and molecular characterization of patients with familial isolated pituitary adenomas (FIPA).. nameOfConference


    QMRO: qmroHref
  • Wilkins S, Choglay AA, Chapple JP et al. (2010). The binding of the molecular chaperone Hsc70 to the prion protein PrP is modulated by pH and copper. nameOfConference


    QMRO: qmroHref
  • Sen Gupta P, Prodromou NV, Chapple JP (2009). Can faulty antennae increase adiposity? The link between cilia proteins and obesity.. nameOfConference


    QMRO: qmroHref
  • Storr HL, Kind B, Parfitt DA et al. (2009). Deficiency of ferritin heavy-chain nuclear import in triple a syndrome implies nuclear oxidative damage as the primary disease mechanism.. nameOfConference


    QMRO: qmroHref
  • Storr HL, Kind B, Parfitt DA et al. (2009). Deficiency of Ferritin Heavy-Chain Nuclear Import in Triple A Syndrome Implies Nuclear Oxidative Damage as the Primary Disease Mechanism. nameOfConference


    QMRO: qmroHref
  • Parfitt DA, Michael GJ, Vermeulen EGM et al. (2009). The ataxia protein sacsin is a functional co-chaperone that protects against polyglutamine-expanded ataxin-1.. nameOfConference


    QMRO: qmroHref
  • Chan LF, Webb TR, Chung T-T et al. (2009). MRAP and MRAP2 are bidirectional regulators of the melanocortin receptor family.. nameOfConference


    QMRO: qmroHref
  • Webb TR, Chan L, Cooray SN et al. (2009). Distinct melanocortin 2 receptor accessory protein domains are required for melanocortin 2 receptor interaction and promotion of receptor trafficking.. nameOfConference


    QMRO: qmroHref
  • Leontiou CA, Gueorguiev M, Hassan S et al. (2009). AIP, a Protein Mutated in Familial Acromegaly, Plays a Role in the Regulation of Cell Proliferation and Shows Cell-Type Specific Subcellular Localisation. nameOfConference


    QMRO: qmroHref
  • Meimaridou E, Gooljar SB, Chapple JP (2009). From hatching to dispatching: the multiple cellular roles of the Hsp70 molecular chaperone machinery.. nameOfConference


    QMRO: qmroHref
  • Chapple JP, Bros-Facer V, Butler R et al. (2008). Focal distortion of the nuclear envelope by huntingtin aggregates revealed by lamin immunostaining. nameOfConference


    QMRO: qmroHref
  • Chung TT, Webb TR, Chan LF et al. (2008). The Majority of Adrenocorticotropin Receptor (Melanocortin 2 Receptor) Mutations Found in Familial Glucocorticoid Deficiency Type 1 Lead to Defective Trafficking of the Receptor to the Cell Surface. nameOfConference


    QMRO: qmroHref
  • Leontiou CA, Gueorguiev M, van der Spuy J et al. (2008). The role of the aryl hydrocarbon receptor-interacting protein gene in familial and sporadic pituitary adenomas.. nameOfConference


    QMRO: qmroHref
  • Cooray SN, Almiro Do Vale I, Leung K-Y et al. (2008). The melanocortin 2 receptor accessory protein exists as a homodimer and is essential for the function of the melanocortin 2 receptor in the mouse y1 cell line.. nameOfConference


    QMRO: qmroHref
  • CHAPPLE JP, Gallo JM, Cooper TA et al. (2007). Expression, localization and Tau Exon 10 splicing activity of the brain RNA-binding protein TNRC4. nameOfConference


    QMRO: qmroHref
  • CHAPPLE JP, Dev A, Anthony K et al. (2007). Expression, localization and tau exon 10 splicing activity of the brain RNA-binding protein TNRC4. nameOfConference


    QMRO: qmroHref
  • Howarth JL, Kelly S, Keasey MP et al. (2007). Hsp40 Molecules That Target to the Ubiquitin-proteasome System Decrease Inclusion Formation in Models of Polyglutamine Disease.. nameOfConference


    QMRO: qmroHref
  • Howarth JL, Kelly S, Keasey MP et al. (2007). Hsp40 molecules that target to the ubiquitin-proteasome system decrease inclusion formation in models of polyglutamine disease. nameOfConference


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  • Hooper C, Chapple JP, Lovestone S et al. (2007). The Notch-1 intracellular domain is found in sub-nuclear bodies in SH-SY5Y neuroblastomas and in primary cortical neurons. nameOfConference


    QMRO: qmroHref
  • Nicoll WS, Botha M, McNamara C et al. (2007). Cytosolic and ER J-domains of mammalian and parasitic origin can functionally interact with DnaK. nameOfConference


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  • Hooper C, Tavassoli M, Chapple JP et al. (2006). TAp73 isoforms antagonize Notch signalling in SH-SY5Y neuroblastomas and in primary neurones. nameOfConference


    QMRO: qmroHref
  • Storr H, Koehler K, Huebner A et al. (2006). A candidate interacting protein for the nuclear pore protein ALADIN: a potential pathogenic mechanism for the triple A syndrome. nameOfConference

    DOI: doi

    QMRO: qmroHref
  • CHAPPLE JP, Adamson P, Matter K et al. (2006). Organization on the plasma membrane of the retinitis pigmentosa protein RP2: investigation of association with detergent-resistant membranes and polarized sorting. nameOfConference


    QMRO: qmroHref
  • Ross AJ, May-Simera H, Eichers ER et al. (2005). Erratum: Corrigendum: Disruption of Bardet-Biedl syndrome ciliary proteins perturbs planar cell polarity in vertebrates. nameOfConference


    QMRO: qmroHref
  • CHAPPLE JP, Fisher S, Tan PL et al. (2005). Disruption of Bardet-Biedl syndrome ciliary proteins perturbs planar cell polarity in vertebrates. nameOfConference


    QMRO: qmroHref
  • CHAPPLE JP, Cheetham ME, Westhoff B et al. (2005). HSJ1 Is a Neuronal Shuttling Factor for the Sorting of Chaperone Clients to the Proteasome. nameOfConference


    QMRO: qmroHref
  • David A, CHAPPLE JP, Cooray S et al. (2005). Mutations in MRAP, encoding a new interacting partner of the ACTH receptor, cause familial glucocorticoid deficiency type 2. nameOfConference


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  • Evans RJ, Chapple JP, Grayson C et al. (2005). Assay and functional analysis of the ARL3 effector RP2 involved in X-linked retinitis pigmentosa.. nameOfConference


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  • CHAPPLE JP, Banerjee R, Gaasenbeek M et al. (2005). Isolation and characterization of murine Cds (CDP-diacylglycerol synthase) 1 and 2. nameOfConference


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  • CHAPPLE JP, Cheetham ME, Van Der SJ et al. (2005). Mechanisms of cell death in rhodopsin retinitis pigmentosa: implications for therapy. nameOfConference


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  • Longshaw VM, Chapple JP, Balda MS et al. (2004). Nuclear translocation of the Hsp70/Hsp90 organizing protein mSTI1 is regulated by cell cycle kinases. nameOfConference


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  • CHAPPLE JP, Holder GE, Egan CA et al. (2004). An atypical phenotype of macular and peripapillary retinal atrophy caused by a mutation in the RP2 gene. nameOfConference


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  • CHAPPLE JP, Cheetham ME, Poopalasundaram S et al. (2004). Neuronal DnaJ proteins HSJ1a and HSJ1b: a role in linking the Hsp70 chaperone machine to the ubiquitin-proteasome system?. nameOfConference


    QMRO: qmroHref
  • CHAPPLE JP, Cheetham ME, Blatch GL et al. (2004). Nuclear translocation of the Hsp70/Hsp90 organizing protein mSTI1 is regulated by cell cycle kinases. nameOfConference

    DOI: doi

    QMRO: qmroHref
  • Chapple JP, Cheetham ME (2003). The chaperone environment at the cytoplasmic face of the endoplasmic reticulum can modulate rhodopsin processing and inclusion formation.. nameOfConference


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  • CHAPPLE JP, Cheetham ME (2003). The Chaperone Environment at the Cytoplasmic Face of the Endoplasmic Reticulum Can Modulate Rhodopsin Processing and Inclusion Formation. nameOfConference


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  • Chapple JP, Hardcastle AJ, Grayson C et al. (2002). Delineation of the plasma membrane targeting domain of the X-linked retinitis pigmentosa protein RP2.. nameOfConference

    DOI: doi

    QMRO: qmroHref
  • van der Spuy J, Chapple JP, Clark BJ et al. (2002). The Leber congenital amaurosis gene product AIPL1 is localized exclusively in rod photoreceptors of the adult human retina.. nameOfConference


    QMRO: qmroHref
  • CHAPPLE JP, Willison KR, Grayson C et al. (2002). In vitro analysis of aminoglycoside therapy for the Arg120stop nonsense mutation in RP2 patients. nameOfConference


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  • CHAPPLE JP, Lewis SA, Willison KR et al. (2002). Localization in the human retina of the X-linked retinitis pigmentosa protein RP2, its homologue cofactor C and the RP2 interacting protein Arl3. nameOfConference


    QMRO: qmroHref
  • CHAPPLE JP, Cheetham ME, Blatch GL et al. (2001). Identification and characterization of a human mitochondrial homologue of the bacterial co-chaperone GrpE. nameOfConference


    QMRO: qmroHref
  • CHAPPLE JP, Grayson C, Saliba RS et al. (2001). Unfolding retinal dystrophies: a role for molecular chaperones?. nameOfConference


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  • Chapple JP, Hardcastle AJ, Grayson C et al. (2000). Mutations in the N-terminus of the X-linked retinitis pigmentosa protein RP2 interfere with the normal targeting of the protein to the plasma membrane. nameOfConference


    QMRO: qmroHref
  • Chapple JP, Hardcastle AJ, Kurzik-Dumke U et al. (1999). Assignment1 of the neuronal cochaperone, HSJ1, to human chromosome bands 2q32→q34 between D2S295 and D2S339 by in situ hybridization and somatic cell and radiation hybrids. nameOfConference


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  • Chapple JP, Smerdon GR, Berry RJ et al. (1998). Seasonal changes in stress-70 protein levels reflect thermal tolerance in the marine bivalve Mytilus edulis L.. nameOfConference


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  • Chapple JP, Smerdon GR, Hawkins AJS (1997). Stress-70 protein induction in Mytilus edulis: Tissue-specific responses to elevated temperature reflect relative vulnerability and physiological function. nameOfConference


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  • Smerdon GR, Chapple JP, Hawkins AJS (1995). The simultaneous immunological detection of four stress-70 protein isoforms in Mytilus edulis. nameOfConference


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Sponsors

Collaborators

Internal

External

  • Professor Martin Knight (School of Engineering and Materials Science, QMUL)
  • Professor Mike Cheetham (UCL Institute of Ophthalmology)
  • Dr Konstantinos Thalassinos (UCL Institute of Structural and Molecular Biology)
  • Dr Paola Giunti (UCL Institute of Neurology)
  • Dr Bernard Brais and Dr Benoit Gentil (Montreal Neurological Institute and Hospital, McGill University Montreal)
  • Francesca Maltecca (Università Vita-Salute San Raffaele, Milan)

News

September 2017
PhD student Lisa Romano wins a highly commended for her poster presentation on ARSACS at the 2nd International Ataxia Research Conference in Pisa, Italy.

July 2017
BBSRC funding awarded for a three-year project to investigate the role of a molecular chaperone Molecular chaperones in the regulation of the intermediate filament cytoskeleton.

June 2017
ARSACS Foundation funded-scientists at Queen Mary University of London, McGill University and Università Vita-Salute San Raffaele have just published a research paper in the journal Human Molecular Genetics. This work describes the cytoskeleton in cells cultured from ARSACS patients. The cytoskeleton is a dynamic network of filaments that pervades the cytoplasm of cells. It acts to regulate cellular shape and internal organisation, while providing the mechanical support that enables cells to divide and move. The researchers have discovered that one component of the cytoskeleton, known as intermediate filaments, has a dramatically altered organisation in skin cells from ARSACS patients. This in turn impacts on the internal organisation of these cells, as well as the machinery they uses to deal with damaged and unwanted components. This research increases knowledge of what may go wrong at the cellular level in ARSACS.

Professor Paul Chapple (lead researcher for this paper) said “We have identified a cytoskeletal phenotype in skin cells from ARSACS patients. This is important as it should be possible to use this cellular phenotype as a readout in assays to screen for drugs that could have potential therapeutic benefit for ARSACS”. 

Teaching

Academic responsibilities

  • Co-Centre Lead, Centre for Endocrinology
  • WHRI Director of Graduate Studies
  • Lecturer for MB BS, PgDip/Msc Endocrinology, BMedSci Molecular Medicine/Molecular Therapeutic, BSc Neuroscience
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