A novel high-throughput immunofluorescence analysis method for quantifying dystrophin intensity in entire transverse sections of Duchenne muscular dystrophy muscle biopsy samples

Valentina Sardone; Matthew Ellis; Silvia Torelli; Lucy Feng; Darren Chambers; Deborah Eastwood; Caroline Sewry; Rahul Phadke; Jennifer E. Morgan; Francesco Muntoni

doi:10.1371/journal.pone.0194540

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Open Access

Peer-reviewed

Research Article

A novel high-throughput immunofluorescence analysis method for quantifying dystrophin intensity in entire transverse sections of Duchenne muscular dystrophy muscle biopsy samples

Valentina Sardone,

Roles Conceptualization, Data curation, Formal analysis, Investigation, Methodology, Project administration, Validation, Writing – original draft

Affiliation Dubowitz Neuromuscular Centre, Molecular Neurosciences Section, Developmental Neuroscience Programme, UCL Great Ormond Street Institute of Child Health, London, United Kingdom

http://orcid.org/0000-0003-2499-9560

⨯
Matthew Ellis,

Roles Data curation, Formal analysis, Investigation, Methodology, Software, Validation, Writing – review & editing

Affiliation Department of Neurodegenerative Diseases, UCL Institute of Neurology, London, United Kingdom
⨯
Silvia Torelli,

Roles Conceptualization, Formal analysis, Validation, Writing – original draft

Affiliation Dubowitz Neuromuscular Centre, Molecular Neurosciences Section, Developmental Neuroscience Programme, UCL Great Ormond Street Institute of Child Health, London, United Kingdom
⨯
Lucy Feng,

Roles Investigation, Methodology, Writing – review & editing

Affiliations Dubowitz Neuromuscular Centre, Molecular Neurosciences Section, Developmental Neuroscience Programme, UCL Great Ormond Street Institute of Child Health, London, United Kingdom, Division of Neuropathology, UCL Institute of Neurology, National Hospital for Neurology and Neurosurgery, UCLH NHS Foundation Trust, London, United Kingdom
⨯
Darren Chambers,

Roles Investigation, Methodology, Writing – review & editing

Affiliations Dubowitz Neuromuscular Centre, Molecular Neurosciences Section, Developmental Neuroscience Programme, UCL Great Ormond Street Institute of Child Health, London, United Kingdom, Division of Neuropathology, UCL Institute of Neurology, National Hospital for Neurology and Neurosurgery, UCLH NHS Foundation Trust, London, United Kingdom
⨯
Deborah Eastwood,

Roles Methodology, Writing – review & editing

Affiliations Department of Orthopaedics, Great Ormond Street Hospital, London, United Kingdom, The Royal National Orthopaedic Hospital, Stanmore, United Kingdom
⨯
Caroline Sewry,

Roles Conceptualization, Investigation, Methodology, Supervision, Writing – review & editing

Affiliations Dubowitz Neuromuscular Centre, Molecular Neurosciences Section, Developmental Neuroscience Programme, UCL Great Ormond Street Institute of Child Health, London, United Kingdom, Wolfson Centre for Inherited Neuromuscular Diseases, RJAH Orthopaedic Hospital, Oswestry, United Kingdom
⨯
Rahul Phadke,

Roles Conceptualization, Investigation, Methodology, Supervision, Writing – review & editing

Affiliations Dubowitz Neuromuscular Centre, Molecular Neurosciences Section, Developmental Neuroscience Programme, UCL Great Ormond Street Institute of Child Health, London, United Kingdom, Division of Neuropathology, UCL Institute of Neurology, National Hospital for Neurology and Neurosurgery, UCLH NHS Foundation Trust, London, United Kingdom
⨯
Jennifer E. Morgan,

Roles Conceptualization, Data curation, Formal analysis, Funding acquisition, Investigation, Methodology, Supervision, Validation, Writing – original draft

Affiliation Dubowitz Neuromuscular Centre, Molecular Neurosciences Section, Developmental Neuroscience Programme, UCL Great Ormond Street Institute of Child Health, London, United Kingdom
⨯
Francesco Muntoni

Roles Conceptualization, Data curation, Formal analysis, Funding acquisition, Investigation, Methodology, Supervision, Validation, Writing – original draft

* E-mail: f.muntoni@ucl.ac.uk

Affiliations Dubowitz Neuromuscular Centre, Molecular Neurosciences Section, Developmental Neuroscience Programme, UCL Great Ormond Street Institute of Child Health, London, United Kingdom, NIHR Great Ormond Street Hospital Biomedical Research Centre, London, United Kingdom
⨯

A novel high-throughput immunofluorescence analysis method for quantifying dystrophin intensity in entire transverse sections of Duchenne muscular dystrophy muscle biopsy samples

Valentina Sardone,
Matthew Ellis,
Silvia Torelli,
Lucy Feng,
Darren Chambers,
Deborah Eastwood,
Caroline Sewry,
Rahul Phadke,
Jennifer E. Morgan,
Francesco Muntoni

Published: March 26, 2018
https://doi.org/10.1371/journal.pone.0194540

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Dystrophin
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Duchenne muscular dystrophy
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Spectrins
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Biopsy
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Muscle proteins
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Muscle fibers
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