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Immunohistological intensity measurements as a tool to assess sarcolemma-associated protein expression

Arechavala-Gomeza, V; Kinali, M; Feng, L; Brown, SC; Sewry, C; Morgan, JE; Muntoni, F; (2010) Immunohistological intensity measurements as a tool to assess sarcolemma-associated protein expression. NEUROPATH APPL NEURO , 36 (4) 265 - 274. 10.1111/j.1365-2990.2009.01056.x. Green open access

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Abstract

Aims: The quantification of protein levels in muscle biopsies is of particular relevance in the diagnostic process of neuromuscular diseases, but is difficult to assess in cases of partial protein deficiency, particularly when information on protein localization is required. The combination of immunohistochemistry and Western blotting is often used in these cases, but is not always possible if the sample is scarce. We therefore sought to develop a method to quantify relative levels of sarcolemma-associated proteins using digitally captured images of immunolabelled sections of skeletal muscle. Methods: To validate our relative quantification method, we labelled dystrophin and other sarcolemmal proteins in transverse sections of muscle biopsies taken from Duchenne muscular dystrophy and Becker muscular dystrophy patients, a manifesting carrier of Duchenne muscular dystrophy and normal controls. Results: Using this method to quantify relative sarcolemmal protein abundance, we were able to accurately distinguish between the different patients on the basis of the relative amount of dystrophin present. Conclusions: This comparative method adds value to techniques that are already part of the diagnostic process and can be used with minimal variation of the standardized protocols, without using extra amounts of valuable biopsy samples. Comparative quantification of sarcolemmal proteins on immunostained muscle sections will be of use to establish both the abundance and localization of the protein. Moreover, it can be applied to assess the efficacy of experimental therapies where only partial restoration or upregulation of the protein may occur.

Type: Article
Title: Immunohistological intensity measurements as a tool to assess sarcolemma-associated protein expression
Open access status: An open access version is available from UCL Discovery
DOI: 10.1111/j.1365-2990.2009.01056.x
Publisher version: http://dx.doi.org/10.1111/j.1365-2990.2009.01056.x
Language: English
Additional information: Copyright 2010 Wiley Blackwell
Keywords: Becker muscular dystrophy, Duchenne muscular dystrophy, genetic therapies, immunohistochemistry, protein quantification, DUCHENNE MUSCULAR-DYSTROPHY, SKELETAL-MUSCLE, LAMININ ALPHA-2, ROD DOMAIN, DEFICIENCY, GENE, MUTATIONS, BIOPSY
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Developmental Neurosciences Dept
URI: https://discovery.ucl.ac.uk/id/eprint/1380290
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