Characterization of lobulated fibers in limb girdle muscular dystrophy type 2A by gene expression profiling

Yoko Keira, Satoru Noguchi, Rumi Kurokawa, Masako Fujita, Narihiro Minami, Yukiko K. Hayashi, Takashi Kato, Ichizo Nishino

    Research output: Contribution to journalArticle

    16 Citations (Scopus)

    Abstract

    Limb girdle muscular dystrophy type 2A (LGMD2A) is caused by mutations in CAPN3, which encodes an intracellular cysteine protease. To elucidate the fundamental molecular changes that may be responsible for the pathological features of LGMD2A, we employed cDNA microarray analysis. We divided LGMD2A muscles into two groups according to specific pathological features: an early-stage group characterized by the presence of active necrosis and a regeneration process and a later-stage group characterized by the presence of lobulated fibers. After comparing the gene expression profiles of the two groups of LGMD2A muscles with control muscles, we identified 29 genes whose mRNA expression profiles were specifically altered in muscles with lobulated fibers. Interestingly, this group included genes that encode actin filament binding and regulatory proteins, such as gelsolin, PDZ and LIM domain 3 (PDLIM3) and troponin I1. Western blot analysis confirmed the upregulation of these proteins. From these results, we propose that abnormal increased expression of actin filament binding proteins may contribute to the changes of the intra-myofiber structures, observed in lobulated fibers in LGMD2A.

    Original languageEnglish
    Pages (from-to)513-521
    Number of pages9
    JournalNeuroscience Research
    Volume57
    Issue number4
    DOIs
    Publication statusPublished - 2007 Apr

    Fingerprint

    Gene Expression Profiling
    Muscles
    Actin Cytoskeleton
    Gelsolin
    PDZ Domains
    Microfilament Proteins
    Troponin I
    Cysteine Proteases
    Microarray Analysis
    Oligonucleotide Array Sequence Analysis
    Transcriptome
    Genes
    Regeneration
    Carrier Proteins
    Necrosis
    Up-Regulation
    Western Blotting
    Limb-girdle muscular dystrophy type 2A
    Messenger RNA
    Mutation

    Keywords

    • Actin filament
    • Calpain 3
    • cDNA microarray
    • Limb girdle muscular dystrophy type 2A
    • Lobulated fiber
    • Myofibril

    ASJC Scopus subject areas

    • Neuroscience(all)

    Cite this

    Keira, Y., Noguchi, S., Kurokawa, R., Fujita, M., Minami, N., Hayashi, Y. K., ... Nishino, I. (2007). Characterization of lobulated fibers in limb girdle muscular dystrophy type 2A by gene expression profiling. Neuroscience Research, 57(4), 513-521. https://doi.org/10.1016/j.neures.2006.12.010

    Characterization of lobulated fibers in limb girdle muscular dystrophy type 2A by gene expression profiling. / Keira, Yoko; Noguchi, Satoru; Kurokawa, Rumi; Fujita, Masako; Minami, Narihiro; Hayashi, Yukiko K.; Kato, Takashi; Nishino, Ichizo.

    In: Neuroscience Research, Vol. 57, No. 4, 04.2007, p. 513-521.

    Research output: Contribution to journalArticle

    Keira, Y, Noguchi, S, Kurokawa, R, Fujita, M, Minami, N, Hayashi, YK, Kato, T & Nishino, I 2007, 'Characterization of lobulated fibers in limb girdle muscular dystrophy type 2A by gene expression profiling', Neuroscience Research, vol. 57, no. 4, pp. 513-521. https://doi.org/10.1016/j.neures.2006.12.010
    Keira, Yoko ; Noguchi, Satoru ; Kurokawa, Rumi ; Fujita, Masako ; Minami, Narihiro ; Hayashi, Yukiko K. ; Kato, Takashi ; Nishino, Ichizo. / Characterization of lobulated fibers in limb girdle muscular dystrophy type 2A by gene expression profiling. In: Neuroscience Research. 2007 ; Vol. 57, No. 4. pp. 513-521.
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