• Open Access

Impaired expression of insulin-like growth factor-1 system in skeletal muscle of amyotrophic lateral sclerosis patients

Authors

  • Christian Lunetta MD,

    Corresponding author
    1. Department of Neurology and Laboratory of Neuroscience, “Dino Ferrari” Center, IRCCS Istituto Auxologico Italiano, Università degli Studi di Milano, Milano, Italy
    2. Neuromuscular Omnicentre, Fondazione Serena Onlus, Niguarda Ca' Granda Hospital, Piazza Ospedale Maggiore 3, Milano, Italy
    • Dino Ferrari Center, University of Milan, IRCCS Foundation Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy
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  • Massimo Serafini BS,

    1. Dino Ferrari Center, University of Milan, IRCCS Foundation Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy
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  • Alessandro Prelle MD, PhD,

    1. U.O. di Neurologia, Dipartimento di Neuroscienze, Azienda Ospedaliera Fatebenefratelli e Oftalmico, Milano, Italy
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  • Paolo Magni MD, PhD,

    1. Department of Endocrinology, Pathophysiology and Applied Biology, Università degli Studi di Milano, Milano, Italy
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  • Elena Dozio PhD,

    1. Department of Human Morphology and Biomedical Sciences “Citt` Studi,” Università degli Studi di Milano, Milano, Italy
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  • Massimiliano Ruscica PhD,

    1. Department of Endocrinology, Pathophysiology and Applied Biology, Università degli Studi di Milano, Milano, Italy
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  • Jenny Sassone PhD,

    1. Department of Neurology and Laboratory of Neuroscience, “Dino Ferrari” Center, IRCCS Istituto Auxologico Italiano, Università degli Studi di Milano, Milano, Italy
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  • Clarissa Colciago PhD,

    1. Department of Neurology and Laboratory of Neuroscience, “Dino Ferrari” Center, IRCCS Istituto Auxologico Italiano, Università degli Studi di Milano, Milano, Italy
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  • Maurizio Moggio MD, PhD,

    1. Dino Ferrari Center, University of Milan, IRCCS Foundation Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy
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  • Massimo Corbo MD, PhD,

    1. Neuromuscular Omnicentre, Fondazione Serena Onlus, Niguarda Ca' Granda Hospital, Piazza Ospedale Maggiore 3, Milano, Italy
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  • Vincenzo Silani MD, PhD

    1. Department of Neurology and Laboratory of Neuroscience, “Dino Ferrari” Center, IRCCS Istituto Auxologico Italiano, Università degli Studi di Milano, Milano, Italy
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Abstract

Introduction:

Adult muscle fibers are a source of growth factors, including insulin-like growth factor-1 (IGF-1). These factors influence neuronal survival, axonal growth, and maintenance of synaptic connections.

Methods:

We investigated the components of the IGF system in skeletal muscle samples obtained from 17 sporadic amyotrophic lateral sclerosis patients (sALS) and 29 control subjects (17 with normal muscle and 12 with denervated muscle unrelated to ALS).

Results:

The muscle expression of IGF-1 and IGF-binding proteins 3, 4, and 5 (IGF-BP3, -4, and -5, respectively), assessed by immunohistochemistry, was differently decreased in sALS compared with both control groups; conversely, IGF-1 receptor β subunit (IGF-1Rβ) was significantly increased. Western blot analysis confirmed the severe reduction of IGF-1, IGF-BP3, and -BP5 with the increment of IGF-1Rβ in sALS.

Conclusion:

In this study we describe the abnormal expression of the IGF-1 system in skeletal muscle of sALS patients that could participate in motor neuron degeneration and should be taken into account when developing treatments with IGF-1. Muscle Nerve, 2012

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