An overexpression of fibroblast growth factor (FGF) and FGF receptor 4 in a severe clinical phenotype of facioscapulohumeral muscular dystrophy

Saito, Akiko; Higuchi, Itsuro; Nakagawa, Masanori; Saito, Mineki; Uchida, Youhei; Inose, Masaru; Kasai, Takefumi; Niiyama, Takahito; Fukunaga, Hidetoshi; Arimura, Kimiyoshi; Osame, Mitsuhiro

doi:10.1002/(sici)1097-4598(200004)23:4<490::aid-mus6>3.0.co;2-k

Cited by 17 publications

(15 citation statements)

References 41 publications

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“…This observation could seem discordant with the known function of FGFs as myoblast differentiation inhibitors (3,31). However FGF1, in contrast to other members of the FGF family, is expressed in dystrophic muscle, suggesting a positive role in the regeneration of skeletal muscle fiber (5,6). The double role of FGF1 as a proliferation activator as well as a differentiation inducer may result from its different functions as an extracellular or intracellular factor (4).…”

Section: Discussionmentioning

confidence: 99%

“…However, intracellular FGF1 has been described as a myoblast differentiation activator (4). Furthermore, although it has been proposed as a negative regulator of muscle development, elevated levels of FGF1 have been observed in regenerating muscle cells of dystrophin-deficient mice (mdx) (5) and in Facioscapulohumoral muscular dystrophy patients (6). Thus, FGF1 is clearly implicated in myogenesis and muscle regeneration, but its role in muscle development is complex and involves non-elucidated mechanisms.…”

Section: Introductionmentioning

confidence: 99%

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Fibroblast growth factor 1 induced during myogenesis by a transcription–translation coupling mechanism

Conte

Ainaoui

Delluc-Clavieres

et al. 2009

Nucleic Acids Research

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Fibroblast growth factor 1 (FGF1) is involved in muscle development and regeneration. The FGF1 gene contains four tissue-specific promoters allowing synthesis of four transcripts with distinct leader regions. Two of these transcripts contain internal ribosome entry sites (IRESs), which are RNA elements allowing mRNA translation to occur in conditions of blockade of the classical cap-dependent mechanism. Here, we investigated the function and the regulation of FGF1 during muscle differentiation and regeneration. Our data show that FGF1 protein expression is induced in differentiating myoblasts and regenerating mouse muscle, whereas siRNA knock-down demonstrated FGF1 requirement for myoblast differentiation. FGF1 induction occurred at both transcriptional and translational levels, involving specific activation of both promoter A and IRES A, whereas global cap-dependent translation was inhibited. Furthermore, we identified, in the FGF1 promoter A distal region, a cis-acting element able to activate the IRES A-driven translation. These data revealed a mechanism of molecular coupling of mRNA transcription and translation, involving a unique process of IRES activation by a promoter element. The crucial role of FGF1 in myoblast differentiation provides physiological relevance to this novel mechanism. This finding also provides a new insight into the molecular mechanisms linking different levels of gene expression regulation.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Fibroblast growth factor 1 induced during myogenesis by a transcription–translation coupling mechanism

Conte

Ainaoui

Delluc-Clavieres

et al. 2009

Nucleic Acids Research

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“…Expression of FGF-1 and 2 is significantly increased in the muscle fibers of facioscapulohumeral muscular dystrophy (FSHD) [31]. Electrically stimulated rat muscles showed a threefold increase of the mRNA levels of both FGF-1 and FGF-2 [32], indicating release during exercise and possible roles in the response of skeletal muscle to work.…”

Section: Introductionmentioning

confidence: 99%

“…In electrically stimulated muscle, expression of FGFR1 was isolated to skeletal muscle fibers, and is doubled over non-stimulated muscles [32]. FGFR-4 expression is significantly increased in the connective tissue of facioscapulohumeral muscular dystrophy (a form of muscular dystrophy) [31]. …”

Section: Introductionmentioning

confidence: 99%

FGFR1 inhibits skeletal muscle atrophy associated with hindlimb suspension

Eash¹,

Olsen

Breur³

et al. 2007

BMC Musculoskelet Disord

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Background: Skeletal muscle atrophy can occur under many different conditions, including prolonged disuse or immobilization, cachexia, cushingoid conditions, secondary to surgery, or with advanced age. The mechanisms by which unloading of muscle is sensed and translated into signals controlling tissue reduction remains a major question in the field of musculoskeletal research. While the fibroblast growth factors (FGFs) and their receptors are synthesized by, and intimately involved in, embryonic skeletal muscle growth and repair, their role maintaining adult muscle status has not been examined.

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“…In this context, it was demonstrated that human muscle cells possess all the intracellular machinery required for antigen processing in the context of MHC I/II presentation [59, 60]. Moreover, presence of fibroblast growth factor (FGF) in dystrophic muscle [61] may on the one hand regulate proliferation of myogenic progenitors [62] but on the other hand lead to expression of the MHC class II receptor HLA-DR, as it has been identified in human mesenchymal stem cells [63]. Aside from MHC molecules, myoblasts can express a nonclassical MHC I molecule, human leukocyte antigen-G (HLA-G), under inflammatory conditions [64].…”

Section: Immunologically Relevant Molecules Expressed By Muscle Cellsmentioning

confidence: 99%

Stem Cell Transplantation for Muscular Dystrophy: The Challenge of Immune Response

Maffioletti

Noviello

English

et al. 2014

BioMed Research International

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Treating muscle disorders poses several challenges to the rapidly evolving field of regenerative medicine. Considerable progress has been made in isolating, characterizing, and expanding myogenic stem cells and, although we are now envisaging strategies to generate very large numbers of transplantable cells (e.g., by differentiating induced pluripotent stem cells), limitations directly linked to the interaction between transplanted cells and the host will continue to hamper a successful outcome. Among these limitations, host inflammatory and immune responses challenge the critical phases after cell delivery, including engraftment, migration, and differentiation. Therefore, it is key to study the mechanisms and dynamics that impair the efficacy of cell transplants in order to develop strategies that can ultimately improve the outcome of allogeneic and autologous stem cell therapies, in particular for severe disease such as muscular dystrophies. In this review we provide an overview of the main players and issues involved in this process and discuss potential approaches that might be beneficial for future regenerative therapies of skeletal muscle.

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An overexpression of fibroblast growth factor (FGF) and FGF receptor 4 in a severe clinical phenotype of facioscapulohumeral muscular dystrophy

Cited by 17 publications

References 41 publications

Fibroblast growth factor 1 induced during myogenesis by a transcription–translation coupling mechanism

Fibroblast growth factor 1 induced during myogenesis by a transcription–translation coupling mechanism

FGFR1 inhibits skeletal muscle atrophy associated with hindlimb suspension

Stem Cell Transplantation for Muscular Dystrophy: The Challenge of Immune Response

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