Prof Paul Chapple

Paul Chapple

Professor of Molecular Cell Biology

Centre for Endocrinology, William Harvey Research Institute
Queen Mary University of London
www.qmul.ac.uk/whri/people/academic-staff/items/chapplepaul.html

Research

Cancer, Neuronal

Cancer Models, Neuronal Models

Interests

Cellular models of neurodegenerative and endocrine disease. Focus on diseases where cellular stress responses are activated and/or protein quality control is disrupted. In vitro models include CRISPR/Cas9 gene edited knockout cell lines, siRNA mediated knockdown cell patient fibroblasts, iPSC. Expertise in molecular cell biology and in particular confocal imaging.

Publications

solid heart iconPublications of specific relevance to Predictive in vitro Models

2025

CRISPRa-Mediated Increase of OPA1 Expression in Dominant Optic Atrophy
Becchi G, Whitehead M, Harvey JP, Sladen PE, Dushti M, Chapple JP, Yu-Wai-Man P, Cheetham ME
International Journal of Molecular Sciences, Mdpi vol. 26 (13) 
02-07-2025

2024

Altered Cellular Metabolism Is a Consequence of Loss of the Ataxia-Linked Protein Sacsin
Perna L, Salsbury G, Dushti M, Smith CJ, Morales V, Bianchi K, Czibik G, Chapple JP
International Journal of Molecular Sciences, Mdpi vol. 25 (24) 
10-12-2024
Network-based modelling reveals cell-type enriched patterns of non-coding RNA regulation during human skeletal muscle remodelling
Mcleod JC, Lim C, Stokes T, Sharif J-A, Zeynalli V, Wiens L, D’Souza AC, Colenso-Semple L et al.
Nar Molecular Medicine, Oxford University Press (Oup) vol. 1 (4) 
01-10-2024
N-SREBP2 Provides a Mechanism for Dynamic Control of Cellular Cholesterol Homeostasis
Ozkan-Nikitaras T, Grzesik DJ, Romano LEL, King PJ
Cells, Mdpi vol. 13 (15) 
25-07-2024

2023

J-domain proteins: From molecular mechanisms to diseases
Marszalek J, De Los Rios P, Cyr D, Mayer MP, Adupa V, Andréasson C, Blatch GL, Braun JEA et al.
Cell Stress and Chaperones, Elsevier vol. 29 (1), 21-33.  
23-12-2023
AlphaFold predicted structure of the Hsp90-like domains of the neurodegeneration linked protein sacsin reveals key residues for ATPase activity
Perna L, Castelli M, Frasnetti E, Romano LEL, Colombo G
Frontiers in Molecular Biosciences, Frontiers vol. 9 
13-01-2023

2022

HSP70-HSP90 Chaperone Networking in Protein-Misfolding Disease
Prodromou C, Aran-Guiu X, Oberoi J, Perna L
Subcellular Biochemistry, Springer Nature vol. 101, 389-425.  
16-12-2022
Multi-omic profiling reveals the ataxia protein sacsin is required for integrin trafficking and synaptic organization
Romano LEL, Aw WY, Hixson KM, Novoselova TV, Havener TM, Howell S, Taylor-Blake B, Hall CL et al.
Cell Reports, Elsevier vol. 41 (5) 
01-11-2022
The immune cell infiltrate in the tumour microenvironment of phaeochromocytomas and paragangliomas
Tufton N, Hearnden RJ, Berney DM, Drake WM, Parvanta L, Chapple JP, Akker SA
Endocrine Related Cancer, Bioscientifica vol. 29 (11), 589-598.  
19-09-2022
Modelling autosomal dominant optic atrophy associated with OPA1 variants in iPSC-derived retinal ganglion cells
Sladen PE, Jovanovic K, Guarascio R, Ottaviani D, Salsbury G, Novoselova T, Chapple JP, Yu-Wai-Man P et al.
Human Molecular Genetics, Oxford University Press (Oup) vol. 31 (20), 3478-3493.  
02-06-2022

2021

Antiproliferative effects of metformin in cellular models of pheochromocytoma
Meireles CG, Lourenço de Lima C, Martins de Paula Oliveira M, Abe da Rocha Miranda R, Yo-Stella Brashaw T, Neves da Silva Guerra E, de Assis Rocha Neves F, Simeoni LA
Molecular and Cellular Endocrinology, Elsevier vol. 539 
09-10-2021
CRISPR-Cas9 correction of OPA1 c.1334G>A: p.R445H restores mitochondrial homeostasis in dominant optic atrophy patient-derived iPSCs
Sladen PE, Perdigão PRL, Salsbury G, Novoselova T, van der Spuy J, Chapple JP, Yu-Wai-Man P, Cheetham ME
Molecular Therapy - Nucleic Acids, Elsevier vol. 26, 432-443.  
19-08-2021
GATA3 induces mitochondrial biogenesis in primary human CD4+ T cells during DNA damage
Callender LA, Schroth J, Carroll EC, Garrod-Ketchley C, Romano LEL, Hendy E, Kelly A
Nature Communications, Nature Research (Part of Springer Nature) 
07-06-2021
Polycystin-2 is required for chondrocyte mechanotransduction and traffics to the primary cilium in response to mechanical stimulation
Thompson C, Mcfie M, Chapple J, BEALES P, Knight M
International Journal of Molecular Sciences, Mdpi Ag 
21-04-2021

2020

Mitochondria form contact sites with the nucleus to couple prosurvival retrograde response
Desai R, East DA, Hardy L, Faccenda D, Rigon M, Crosby J, Alvarez MS, Singh A et al.
Science Advances, American Association For The Advancement of Science (Aaas) vol. 6 (51) 
18-12-2020
Corrigendum to: The co-chaperone and reductase ERdj5 facilitates rod opsin biogenesis and quality control
Athanasiou D, Bevilacqua D, Aguila M, McCulley C, Iwawaki T, Chapple JP
Human Molecular Genetics, Oxford University Press (Oup) vol. 29 (19), 3338-3339.  
28-09-2020

2019

Mechanical loading inhibits cartilage inflammatory signalling via an HDAC6 and IFT-dependent mechanism regulating primary cilia elongation
Fu S, Thompson CL, Ali A, Wang W, Chapple JP, Mitchison HM, Beales PL, Wann AKT et al.
Osteoarthritis and Cartilage, Elsevier vol. 27 (7), 1064-1074.  
25-03-2019
Oncometabolite induced primary cilia loss in pheochromocytoma
O’Toole SM, Watson DS, Novoselova TV, Romano LEL, King PJ, Bradshaw TY, Thompson CL, Knight MM et al.
Endocrine Related Cancer vol. 26 (1), 165-180.  
01-01-2019
Mitochondria Form Contact Sites with the Nucleus to Couple Pro-Survival Retrograde Response
Desai R, East DA, Hardy L, Crosby J, Rigon M, Faccenda D, Alvarez MS, Singh A et al.
In Ssrn Electronic Journal 
01-01-2019

2018

Can novel stem cell models help unpick the pathogenesis of the Triple A syndrome?
Costa ARD, Qarin S, Bradshaw T, Watson D, Prasad R, Metherell LA, Barnes MR, Skarnes W et al.
Endocrine Abstracts, Bioscientifica 
18-10-2018
Sacsin, mutated in the ataxia ARSACS, regulates intermediate filament assembly and dynamics
Gentil BJ, Lai G-T, Menade M, Minotti S, Chapple J-P, Brais B
The Faseb Journal, Wiley vol. 33 (2), 2982-2994.  
17-10-2018
A Novel Stem Cell Model for the Triple a Syndrome
Da Costa AR, Qarin S, Bradshaw TY, Watson D, Prasad R, Barnes MR, Metherell LA, Chapple JP et al.
57th Annual Meeting of the European Society for Paediatric Endocrinology (ESPE) Athens, Greece 27 Sep 2018 - 29 Sep 2018. 
14-09-2018
Optical coherence tomography in autosomal recessive spastic ataxia of Charlevoix-Saguenay
Parkinson MH, Bartmann AP, Clayton LMS, Nethisinghe S, Pfundt R, Reilly MM, Wood NJ
Brain, Oxford University Press (Oup) vol. 141 (4), 989-999.  
12-03-2018

2017

Novel evidence implies that ALADIN, the triple A syndrome gene product is involved in mitochondrial physiology
Da CAR, Meimaridou E, Prasad R, Metherell LA, Chapple JP, Storr HL
Endocrine Abstracts, Bioscientifica 
30-10-2017
Chondrocyte expansion is associated with loss of primary cilia and disrupted hedgehog signalling.
Thompson CL, Plant JC, Wann AK, Bishop CL, Novak P, Mitchison HM, Beales PL, Chapple JP et al.
Eur Cell Mater vol. 34, 128-141.  
20-09-2017
Topography of calcium phosphate ceramics regulates primary cilia length and TGF receptor recruitment associated with osteogenesis.
Zhang J, Dalbay MT, Luo X, Vrij E, Barbieri D, Moroni L, de Bruijn JD, van Blitterswijk CA et al.
Acta Biomater vol. 57, 487-497.  
15-07-2017
Altered organisation of the intermediate filament cytoskeleton and relocalisation of proteostasis modulators in cells lacking the ataxia protein sacsin
CHAPPLE JP
Human Molecular Genetics, Oxford University Press 
23-05-2017
Arl3 and RP2 regulate the trafficking of ciliary tip kinesins.
Schwarz N, Lane A, Jovanovic K, Parfitt DA, Aguila M, Thompson CL, da Cruz L, Coffey PJ
Hum Mol Genet, Oxford University Press vol. 26 (13), 2480-2492.  
21-04-2017
Arl3 and RP2 regulate the trafficking of ciliary tip kinesins. (CORRIGENDUM)
Schwarz N, Lane A, Jovanovic K, Parfitt DA, Aguila M, da Cruz L, Coffey PJ, Hardcastle AJ
Hum Mol Genet, Oxford University Press vol. 26 (17), 3451-3451.  
21-04-2017
Polycystins are required for primary cilia-mediated mechanotransduction in chondrocytes
Thompson CL, Chapple JP, Beales PL, Mitchison HM, Ramachandran M, Knight MM
 
01-01-2017

2016

Primary cilia: a link between hormone signalling and endocrine-related cancers?
O'Toole SM, Chapple JP
Biochemical Society Transactions, Portland Press vol. 44 (5), 1227-1234.  
15-10-2016
A reduction in Drp1 mediated fission compromises mitochondrial health in autosomal recessive spastic ataxia of Charlevoix Saguenay
CHAPPLE JP, Bradshaw TY, Romano LEL, Nethisinghe S, Abeti R, Michael GJ, Giunti P
Human Molecular Genetics, Oxford University Press (Oup): Policy B - Oxford Open Option B vol. 25 (15), 3232-3244.  
10-06-2016
The role of primary cilia in the molecular pathogenesis of phaeochromocytoma
O'Toole S, Srirangalingam U, Drake W
Endocrine Abstracts, Bioscientifica 
13-05-2016
Chondrocyte dedifferentiation down regulates mechano-responsiveness and hedgehog signalling associated with changes in primary cilia structure
Kelly T-AN, Thompson CL, Tan E, Wann AK, Thorpe SD, Chapple JP, Hung CT, Knight MM
World Congress of the Osteoarthritis-Research-Society-International (OARSI) on Osteoarthritis 31 Mar 2016 - 3 Apr 2016. 
01-04-2016

2015

Hedgehog signalling does not stimulate cartilage catabolism and is inhibited by Interleukin-1β
Thompson CL, Patel R, Kelly T-AN, Wann AKT, Hung CT, Chapple JP, Knight MM
Arthritis Research & Therapy vol. 17 (1) 
24-12-2015
Adipogenic Differentiation of hMSCs is Mediated by Recruitment of IGF‐1r Onto the Primary Cilium Associated With Cilia Elongation
Dalbay MT, Thorpe SD, Connelly JT, Chapple JP, Knight MM
Stem Cells, Oxford University Press (Oup) vol. 33 (6), 1952-1961.  
21-05-2015
Molecular and functional studies of retinal degeneration as a clinical presentation of SACS-related disorder
Blumkin L, Bradshaw T, Michelson M, Kopler T, Dahari D, Lerman-Sagie T
European Journal of Paediatric Neurology, Elsevier vol. 19 (4), 472-476.  
03-03-2015
Competitive interactions between hedgehog and cytokine signalling: crosstalk at the chondrocyte primary cilium?
Thompson CL, Wann AKT, Chapple JP, Poole CA
International Journal of Experimental Pathology vol. 96 (2), A8-A8.  
01-01-2015

2014

The co-chaperone and reductase ERdj5 facilitates rod opsin biogenesis and quality control.
Athanasiou D, Bevilacqua D, Aguila M, McCulley C, Kanuga N, Iwawaki T
Hum Mol Genet vol. 23 (24), 6594-6606.  
15-12-2014
The Role of HSP70 and Its Co-chaperones in Protein Misfolding, Aggregation and Disease
Duncan EJ, Cheetham ME, Chapple JP
Subcellular Biochemistry, Springer Nature vol. 78, 243-273.  
09-12-2014
The primary cilium influences interleukin-1β-induced NFκB signalling by regulating IKK activity.
Wann AKT, Chapple JP, Knight MM
Cell Signal vol. 26 (8), 1735-1742.  
01-08-2014
Primary cilia disassembly down-regulates mechanosensitive hedgehog signalling: a feedback mechanism controlling ADAMTS-5 expression in chondrocytes.
Thompson CL, Chapple JP, Knight MM
Osteoarthritis Cartilage vol. 22 (3), 490-498.  
01-03-2014
Primary cilia disassembly down regulates mechanosensitive hedgehog signalling: a feedback mechanism controlling ADAMTS-5 expression in chondrocytes
Thompson CL, Chapple JP
 
01-01-2014
The chondrocyte primary cilium is required for IL-1 induced NF-κβ signalling
Wann AKT, Chapple JP, Knight MM
 
01-01-2014
The chondrocyte cilium; a locality for HIF regulation in inflammatory signalling
Wann AKT, Thompson CL, Chapple JP
 
01-01-2014

2013

Interleukin-1β sequesters hypoxia inducible factor 2α to the primary cilium.
Wann AK, Thompson CL, Chapple JP, Knight MM
Cilia vol. 2 (1) 
13-12-2013
Familial glucocorticoid deficiency: New genes and mechanisms.
Meimaridou E, Hughes CR, Kowalczyk J, Guasti L, Chapple JP, King PJ
Mol Cell Endocrinol vol. 371 (1-2), 195-200.  
22-05-2013
Familial glucocorticoid deficiency: New genes and mechanisms
Meimaridou E, Hughes CR, Kowalczyk J, Guasti L, Chapple JP
Molecular and Cellular Endocrinology vol. 371 (1-2), 195-200.  
22-05-2013

2012

Heat shock induces rapid resorption of primary cilia
Prodromou NV, Thompson CL, Osborn DPS, Cogger KF, Ashworth R, Knight MM, Beales PL, Chapple JP
Development, The Company of Biologists vol. 139 (24), e2408-e2408.  
15-12-2012
Heat shock induces rapid resorption of primary cilia
Prodromou NV, Thompson CL, Osborn DPS, Cogger KF, Ashworth R, Knight MM, Beales PL, Chapple JP
Journal of Cell Science vol. 125 (18), 4297-4305.  
01-12-2012
Heat-shock induces rapid resorption of primary cilia
Thompson C, Prodromou N, Osborn D, Ashworth R, Knight M, Beales P, Chapple J
Cilia, Springer Nature vol. 1 (Suppl 1) 
01-11-2012
Mechanical strain disrupts primary cilia structure and modulates hedgehog signalling in adult chondrocytes
Thompson C, Chapple J, Knight M
Cilia, Springer Nature vol. 1 (Suppl 1) 
01-11-2012
BiP prevents rod opsin aggregation.
Athanasiou D, Kosmaoglou M, Kanuga N, Novoselov SS, Paton AW
Mol Biol Cell vol. 23 (18), 3522-3531.  
01-09-2012
Somatostatin analogs modulate AIP in somatotroph adenomas: the role of the ZAC1 pathway.
Chahal HS, Trivellin G, Leontiou CA, Alband N, Fowkes RC, Tahir A, Igreja SC, Chapple JP et al.
J Clin Endocrinol Metab vol. 97 (8), E1411-E1420.  
01-08-2012
Mutations in NNT encoding nicotinamide nucleotide transhydrogenase cause familial glucocorticoid deficiency.
Meimaridou E, Kowalczyk J, Guasti L, Hughes CR, Wagner F, Nürnberg P, Banerjee R
Nat Genet vol. 44 (7), 740-742.  
27-05-2012
002 Detecting retinal changes in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) using optical coherence tomography (OCT)
Giunti P, Nethisinghe S, Clayton LM, Vermeer S, Chapple JP
 
09-02-2012
MECHANICAL STRAIN STIMULATES HEDGEHOG SIGNALLING IN ADULT ARTICULAR CHONDROCYTES AND REDUCES PRIMARY CILIA LENGTH
Thompson CL, Chapple JP
 
01-01-2012
Mitochondrial dysfunction and Purkinje cell loss in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS)
Girard M, Lariviere R, Parfitt DA, Deane EC, Gaudet R, Blondeau F, Prenosil G, Duchen MR et al.
Proceedings of The National Academy of Sciences of The United States of America vol. 109 (5), 1661-1666.  
01-01-2012
Mechanical strain reduces primary cilia length and stimulates hedgehog signalling in adult chondrocytes
Thompson CL, Chapple JP
 
01-01-2012
Retinal changes in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) and other genetic ataxias
Parkinson MH, Nethisinghe S, Clayton L, Chapple JP, Reilly M
 
01-01-2012
Hsp70 Chaperone Systems in Vesicular Trafficking
Chapple JP, Parfitt DA
In Cellular Trafficking of Cell Stress Proteins in Health and Disease, Springer Nature 87-101.  
01-01-2012
Heat-shock induces rapid resorption of primary cilia
Thompson CL, Prodromou NV, Osborn DP, Ashworth R, Knight MM, Beales PL, Chapple JP
Cilia vol. 1 (Suppl 1), P52-P52.  
01-01-2012

2011

The cytosolic chaperone Hsc70 promotes traffic to the cell surface of intracellular retained melanocortin-4 receptor mutants.
Meimaridou E, Gooljar SB, Ramnarace N, Clark AJL
Mol Endocrinol vol. 25 (9), 1650-1660.  
01-09-2011
Retinal Imaging in Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay
Nethisinghe S, Clayton L, Vermeer S, Chapple JP, Reilly M, Bremner F
Neuro-Ophthalmology vol. 35 (4), 197-201.  
01-08-2011

2010

Characterization of aryl hydrocarbon receptor interacting protein (AIP) mutations in familial isolated pituitary adenoma families.
Igreja S, Chahal HS, King P, Bolger GB, Srirangalingam U, Guasti L, Gueorguiev M, Guegan K et al.
Hum Mutat vol. 31 (8), 950-960.  
01-08-2010
Clinical, genetic and molecular characterization of patients with familial isolated pituitary adenomas (FIPA).
Chahal HS, Chapple JP, Grossman AB
Trends Endocrinol Metab vol. 21 (7), 419-427.  
01-07-2010
The binding of the molecular chaperone Hsc70 to the prion protein PrP is modulated by pH and copper
Wilkins S, Choglay AA, Chapple JP, van der Spuy J, Rhie A
The International Journal of Biochemistry & Cell Biology, Elsevier vol. 42 (7), 1226-1232.  
29-04-2010

2009

Deficiency of ferritin heavy-chain nuclear import in triple a syndrome implies nuclear oxidative damage as the primary disease mechanism.
Storr HL, Kind B, Parfitt DA, Chapple JP, Lorenz M, Koehler K, Huebner A, Clark AJL
Mol Endocrinol vol. 23 (12), 2086-2094.  
01-12-2009
Can faulty antennae increase adiposity? The link between cilia proteins and obesity.
Sen Gupta P, Prodromou NV
J Endocrinol vol. 203 (3), 327-336.  
01-12-2009
Deficiency of Ferritin Heavy-Chain Nuclear Import in Triple A Syndrome Implies Nuclear Oxidative Damage as the Primary Disease Mechanism
Storr HL, Kind B, Parfitt DA, Chapple JP, Lorenz M, Koehler K, Huebner A, Clark AJL
The Journal of Clinical Endocrinology & Metabolism, The Endocrine Society vol. 94 (11), 4626-4626.  
01-11-2009
The ataxia protein sacsin is a functional co-chaperone that protects against polyglutamine-expanded ataxin-1.
Parfitt DA, Michael GJ, Vermeulen EGM, Prodromou NV, Webb TR, Gallo J-M, Cheetham ME
Hum Mol Genet vol. 18 (9), 1556-1565.  
01-05-2009
MRAP and MRAP2 are bidirectional regulators of the melanocortin receptor family.
Chan LF, Webb TR, Chung T-T, Meimaridou E, Cooray SN, Guasti L, Chapple JP, Egertová M et al.
Proc Natl Acad Sci U S A vol. 106 (15), 6146-6151.  
14-04-2009
Distinct melanocortin 2 receptor accessory protein domains are required for melanocortin 2 receptor interaction and promotion of receptor trafficking.
Webb TR, Chan L, Cooray SN, Chapple JP
Endocrinology vol. 150 (2), 720-726.  
01-02-2009
AIP, a Protein Mutated in Familial Acromegaly, Plays a Role in the Regulation of Cell Proliferation and Shows Cell-Type Specific Subcellular Localisation
Leontiou CA, Gueorguiev M, Hassan S, van der Spuy J, Lolli F, Stolbrink M, Christian HC, Wray J et al.
 
01-01-2009
From hatching to dispatching: the multiple cellular roles of the Hsp70 molecular chaperone machinery.
Meimaridou E, Gooljar SB, Chapple JP
J Mol Endocrinol vol. 42 (1), 1-9.  
01-01-2009

2008

Focal distortion of the nuclear envelope by huntingtin aggregates revealed by lamin immunostaining
Chapple JP, Bros-Facer V
Neurosci Lett vol. 447 (2-3), 172-174.  
12-12-2008
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.
Chung TT, Webb TR, Chan LF, Cooray SN, Metherell LA
J Clin Endocrinol Metab vol. 93 (12), 4948-4954.  
01-12-2008
The role of the aryl hydrocarbon receptor-interacting protein gene in familial and sporadic pituitary adenomas.
Leontiou CA, Gueorguiev M, van der Spuy J, Quinton R, Lolli F, Hassan S, Chahal HS, Igreja SC et al.
J Clin Endocrinol Metab vol. 93 (6), 2390-2401.  
01-06-2008
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.
Cooray SN, Almiro Do Vale I, Leung K-Y, Webb TR, Chapple JP, Egertová M, Cheetham ME, Elphick MR et al.
Endocrinology vol. 149 (4), 1935-1941.  
01-04-2008

2007

Expression, localization and Tau Exon 10 splicing activity of the brain RNA-binding protein TNRC4
CHAPPLE JP, Gallo JM, Cooper TA, Dev A
Human Molecular Genetics vol. 16 (22), 2760-2769.  
01-11-2007
Expression, localization and tau exon 10 splicing activity of the brain RNA-binding protein TNRC4
CHAPPLE JP, Dev A, Anthony K, Cooper TA
Human Molecular Genetics vol. 16 (22), 2760-2769.  
01-11-2007
Hsp40 molecules that target to the ubiquitin-proteasome system decrease inclusion formation in models of polyglutamine disease
Howarth JL, Kelly S, Keasey MP, Glover CPJ, Lee YB, Mitrophanous K, Chapple JP, Gallo JM
Mol Ther vol. 15 (6), 1100-1105.  
01-06-2007
Hsp40 Molecules That Target to the Ubiquitin-proteasome System Decrease Inclusion Formation in Models of Polyglutamine Disease.
Howarth JL, Kelly S, Keasey MP, Glover C, Mitrophanous K, Cheetham ME
Mol Ther vol. 15 (6), 1100-1105.  
01-06-2007
The Notch-1 intracellular domain is found in sub-nuclear bodies in SH-SY5Y neuroblastomas and in primary cortical neurons
Hooper C, Chapple JP, Lovestone S
Neurosci Lett vol. 415 (2), 135-139.  
26-03-2007
Cytosolic and ER J-domains of mammalian and parasitic origin can functionally interact with DnaK
Nicoll WS, Botha M, McNamara C, Schlange M, Pesce ER, Boshoff A, Ludewig MH, Zimmermann R et al.
Int J Biochem Cell B vol. 39 (4), 736-751.  
01-01-2007

2006

TAp73 isoforms antagonize Notch signalling in SH-SY5Y neuroblastomas and in primary neurones
Hooper C, Tavassoli M, Chapple JP, Uwanogho D, Melino G, Lovestone S
J Neurochem vol. 99 (3), 989-999.  
01-11-2006
A candidate interacting protein for the nuclear pore protein ALADIN: a potential pathogenic mechanism for the triple A syndrome
Storr H, Koehler K, Huebner A, Chapple JP, Clark AJL
Horm Res vol. 65, 16-16.  
01-01-2006
Organization on the plasma membrane of the retinitis pigmentosa protein RP2: investigation of association with detergent-resistant membranes and polarized sorting
CHAPPLE JP, Adamson P, Matter K, Hardcastle AJ, Grayson C
Biochem J vol. 372 (pt 2), 427-433.  
01-01-2006

2005

Erratum: Corrigendum: Disruption of Bardet-Biedl syndrome ciliary proteins perturbs planar cell polarity in vertebrates
Ross AJ, May-Simera H, Eichers ER, Kai M, Hill J, Jagger DJ, Leitch CC, Chapple JP et al.
Nature Genetics, Springer Nature vol. 37 (12), 1381-1381.  
01-12-2005
Disruption of Bardet-Biedl syndrome ciliary proteins perturbs planar cell polarity in vertebrates
CHAPPLE JP, Fisher S, Tan PL, Phillips HM, Leitch CC, Eliot MM, Copp AJ, Katsanis N et al.
Nature Genetics vol. 37 (10), 1135-1140.  
01-10-2005
HSJ1 Is a Neuronal Shuttling Factor for the Sorting of Chaperone Clients to the Proteasome
CHAPPLE JP, Westhoff B, Hohfeld J
Current Biology vol. 15 (11), 1058-1064.  
01-06-2005
Mutations in MRAP, encoding a new interacting partner of the ACTH receptor, cause familial glucocorticoid deficiency type 2.
Metherell LA, Chapple JP, Cooray S, David A, Becker C, Rüschendorf F, Naville D, Begeot M
Nat Genet vol. 37 (2), 166-170.  
01-02-2005
Mechanisms of cell death in rhodopsin retinitis pigmentosa: implications for therapy
CHAPPLE JP, Van Der SJ, Mendes HF
Trends Mol Med vol. 11 (4), 177-185.  
01-01-2005
Isolation and characterization of murine Cds (CDP-diacylglycerol synthase) 1 and 2
CHAPPLE JP, Banerjee R, Gaasenbeek M, Ocaka L
Gene vol. 356, 19-31.  
01-01-2005
Assay and functional analysis of the ARL3 effector RP2 involved in X-linked retinitis pigmentosa.
Evans RJ, Chapple JP, Grayson C
Methods Enzymol vol. 404, 468-480.  
01-01-2005

2004

Nuclear translocation of the Hsp70/Hsp90 organizing protein mSTI1 is regulated by cell cycle kinases
Longshaw VM, Chapple JP, Balda MS, Blatch GL
Journal of Cell Science, The Company of Biologists vol. 117 (5), 701-710.  
15-02-2004
An atypical phenotype of macular and peripapillary retinal atrophy caused by a mutation in the RP2 gene
CHAPPLE JP, Holder GE, Egan CA, Grayson C, Ebenezer ND
Br. J Opthalmol vol. 88 (4), 528-532.  
01-01-2004
Neuronal DnaJ proteins HSJ1a and HSJ1b: a role in linking the Hsp70 chaperone machine to the ubiquitin-proteasome system?
CHAPPLE JP, Cheetham ME, Van Der SJ
Biochem Soc Trans vol. 32 (pt4), 640-642.  
01-01-2004
Nuclear translocation of the Hsp70/Hsp90 organizing protein mSTI1 is regulated by cell cycle kinases
CHAPPLE JP, Cheetham ME, Blatch GL
J Cell Sci vol. 117 (pt 5), 701-710.  
01-01-2004

2003

The chaperone environment at the cytoplasmic face of the endoplasmic reticulum can modulate rhodopsin processing and inclusion formation.
Chapple JP, Cheetham ME
J Biol Chem vol. 278 (21), 19087-19094.  
23-05-2003
The Chaperone Environment at the Cytoplasmic Face of the Endoplasmic Reticulum Can Modulate Rhodopsin Processing and Inclusion Formation
CHAPPLE JP, Cheetham ME
Journal of Biological Chemistry vol. 278 (21), 19087-19094.  
01-05-2003

2002

Delineation of the plasma membrane targeting domain of the X-linked retinitis pigmentosa protein RP2.
Chapple JP, Hardcastle AJ, Grayson C, Cheetham ME
Invest Ophthalmol Vis Sci vol. 43 (6), 2015-2020.  
01-06-2002
The Leber congenital amaurosis gene product AIPL1 is localized exclusively in rod photoreceptors of the adult human retina.
van der Spuy J, Chapple JP, Clark BJ, Sethi CS
Hum Mol Genet vol. 11 (7), 823-831.  
01-04-2002
In vitro analysis of aminoglycoside therapy for the Arg120stop nonsense mutation in RP2 patients
CHAPPLE JP, Willison KR, Grayson C
J Med Genet vol. 39 (1), 62-67.  
01-01-2002
Localization in the human retina of the X-linked retinitis pigmentosa protein RP2, its homologue cofactor C and the RP2 interacting protein Arl3
CHAPPLE JP, Lewis SA, Willison KR, Bartolini F, Bhamidipati A
Hum Mol Genet vol. 11 (24), 3065-3074.  
01-01-2002

2001

Identification and characterization of a human mitochondrial homologue of the bacterial co-chaperone GrpE
CHAPPLE JP, Cheetham ME, Choglay AA
Gene vol. 267 (1), 125-134.  
01-01-2001
Unfolding retinal dystrophies: a role for molecular chaperones?
CHAPPLE JP, Grayson C, Saliba RS, Hardcastle AJ
Trends Mol Med vol. 7 (9), 414-421.  
01-01-2001

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
Chapple JP, Hardcastle AJ, Grayson C, Cheetham ME
Human Molecular Genetics, Oxford University Press (Oup) vol. 9 (13), 1919-1926.  
12-08-2000

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
Chapple JP, Hardcastle AJ, Kurzik-Dumke U, Collier DA
Cytogenetic and Genome Research, Karger Publishers vol. 86 (1), 62-63.  
01-01-1999

1998

Seasonal changes in stress-70 protein levels reflect thermal tolerance in the marine bivalve Mytilus edulis L.
Chapple JP, Smerdon GR, Hawkins AJS
Journal of Experimental Marine Biology and Ecology, Elsevier vol. 229 (1), 53-68.  
01-10-1998

1997

Stress-70 protein induction in Mytilus edulis: Tissue-specific responses to elevated temperature reflect relative vulnerability and physiological function
Chapple JP, Smerdon GR, Hawkins AJS
Journal of Experimental Marine Biology and Ecology, Elsevier vol. 217 (2), 225-235.  
01-10-1997

1995

The simultaneous immunological detection of four stress-70 protein isoforms in Mytilus edulis
Smerdon GR, Chapple JP
Marine Environmental Research, Elsevier vol. 40 (4), 399-407.  
01-01-1995

Biallelic Loss of Molecular Chaperone Molecule AIP Results in a Novel Severe Multisystem Disease Defined by Defective Proteostasis
Korbonits M, Wang X, Barry S, Lim CT, Suleyman O, De Tito S, Uddin N, Vignola ML et al.
In Biorxiv 
Network-based modelling reveals cell-type enriched patterns of non-coding RNA regulation during human skeletal muscle remodelling
Mcleod JC, Lim C, Stokes T, Sharif J-A, Zeynalli V, Wiens L, D’Souza AC, Colenso-Semple L et al.
In Biorxiv 
Mitochondria form contact sites with the nucleus to couple pro-survival retrograde response
Desai R, East DA, Hardy L, Crosby J, Rigon M, Faccenda D, Alvarez MS, Singh A et al.
In Biorxiv 
GATA3 controls mitochondrial biogenesis in primary human CD4+ T cells during DNA damage
Callender LA, Schroth J, Carroll EC, Romano LEL, Hendy E, Kelly A, Lavender P, Akbar AN et al.
In Biorxiv 
The ataxia protein sacsin is required for integrin trafficking and synaptic organization
Romano LEL, Aw WY, Hixson KM, Novoselova TV, Havener TM, Howell S, Hall CL, Xing L et al.
In Biorxiv 
AlphaFold predicted structure of the Hsp90-like domains of the neurodegeneration linked protein sacsin reveals key residues for ATPase activity
Perna L, Romano LEL, Prodromou C, Chapple JP
In Biorxiv 

Grants

solid heart iconGrants of specific relevance to Predictive in vitro Models
bullet iconBarts Charity DTP
Amrita Ahluwalia and Paul Chapple
£4,858,360 Barts and the London Charity
01-10-2025 - 30-09-2034
bullet iconUnderstanding the molecular pathology of hyperinsulinaemic hypoglycaemia and neurodegeneration in children with DNAJC3 mutations
Paul Chapple
£138,659 Daphne Jackson Trust, the
22-04-2025 - 21-04-2028
bullet iconUnderstanding trafficking defects in ARSACS to identify therapeutic targets for disease
Paul Chapple and James Timmons
£119,180 ARSACS Ataxia Charlevoix-Saguenay Foundation (Canada)
01-10-2024 - 30-09-2027
bullet iconReducing morbidity and mortality in succinate dehdrogenase gene mutation carriers
Paul Chapple and William Drake
£618,735 Barts and the London Charity
01-08-2022 - 28-08-2026
bullet iconCellular stress responses in SDH disease
Paul Chapple
£136,010 Paradifference Foundation, The
01-10-2021 - 01-12-2026

bullet iconUnveil a novel pathogenic conduit by characterising the sites of contact between nucleus and mitochondria
Michelangelo Campanella, Paul Chapple and Mark Caulfield
£74,964 Barts and the London Charity
01-04-2024 - 30-09-2025
bullet iconMetabolic rewiring in cellular models of ARSACS
Paul Chapple
£75,428 Ataxia Charlevoix-Saguenay Foundation (Canada)
01-10-2022 - 30-09-2025
bullet iconTargeting mitochondrial dysfunction in cellular models of SDH disease
Paul Chapple and Eugenie Su-Yien Lim
£60,609 MRC Medical Research Council
07-02-2022 - 06-02-2025
bullet iconDevelopment and characterisation of novel CRISPR/Cas9 KO cell lines of FH and SDHAF2 as models of PPGL
Grace Salsbury and Paul Chapple
£10,000 Society for Endocrinology
01-07-2021 - 31-07-2023
bullet iconSuper-Resolution Microscopy of live cells in 3D
Viji Draviam Sastry, Conrad Mullineaux, Vidya Darbari, Lakxmi Subramanian, Fabrice Caudron, Richard Pickersgill, Guy Hanke, Alexander Ruban, Peter Thorpe, Christopher Duffy, Jose Martin Duran, John Connelly, Peter McCormick, Paul Chapple, Vardhman Rakyan, Tyson Sharp, Susana Godinho and David Lee
£369,201 BBSRC Biotechnology and Biological Sciences Research Council
01-12-2020 - 31-03-2022
bullet iconIs ARSACS a Tauopathy?
Paul Chapple
£98,481 ARSACS Ataxia Charlevoix-Saguenay Foundation (Canada)
01-02-2020 - 31-01-2024
bullet iconDesign and test the feasibility of a novel smartphone growth monitoring app
Helen Storr, Anne Hsu, Leo Dunkel and Paul Chapple
£104,886 Merck
01-10-2019 - 31-08-2022
bullet iconDNA-repair mechanisms as a therapeutic target for SDH dependent phaeochromocytoma and paraganglioma
Paul Chapple and William Drake
£160,000 Barts and the London Charity
01-04-2019 - 06-02-2022
bullet iconLeaf Expression endocrinology studies
Paul Chapple and Peter McCormick
£5,470 William Harvey Research Limited
01-04-2019 - 31-12-2020
bullet iconMolecular chaperones in the regulation of the intermediate filament cytoskeleton
Paul Chapple
£373,022 BBSRC Biotechnology and Biological Sciences Research Council
04-01-2018 - 03-01-2021
bullet iconDisrupted Protein Homeostatis in Autosomal Reccessive
Paul Chapple
£20,000 Ataxia UK
01-10-2016 - 30-09-2019
bullet iconDisrupted Protein Homeostatis in Autosomal Reccessive Spastic Ataxia
Paul Chapple
£56,896 Ataxia UK
01-10-2016 - 01-10-2020
bullet iconA core confocal microscopy facility for the William Harvey Research Institute
Paul Chapple
£385,032 Barts and the London Charity
01-11-2015 - 31-10-2019