Trueb Beat, Steinberg Florian
Department of Clinical Research; University of Bern; Bern, Switzerland.
Commun Integr Biol. 2011 May;4(3):287-90. doi: 10.4161/cib.4.3.14892.
FGFRL1 is the fifth member of the fibroblast growth factor receptor (FGFR) family. Similar to the other members, it harbors three Ig loops in its extracellular domain, but in contrast to the other receptors, it lacks the intracellular protein tyrosine kinase domain that would be required for signaling by transphosphorylation. FGFRL1 is mainly found in the musculoskeletal system, where it appears to inhibit cell proliferation but to induce cell adhesion and differentiation. Mice with a targeted disruption of the FGFRL1 gene die during birth due to a malformed diaphragm muscle, which is not strong enough to inflate the lungs after birth. Expression of FGFRL1 is highly upregulated during the differentiation of myoblasts to multinucleated myotubes, suggesting an important role for FGFRL1 in cell-cell fusion. Recently we showed that FGFRL1 does indeed induce fusion of cultured cells into large syncytia. A reporter gene assay demonstrated that the third Ig domain and the transmembrane domain of FGFRL1 are both necessary and sufficient to fuse CHO cells into syncytia comprising several hundred nuclei. At the contact site, the fusing cells reveal a peculiar net-like structure with pores of about 1 µm diameter. It is possible that these structures represent membrane areas with fusion pores that set in motion the cell-cell fusion process. FGFRL1 is the first mammalian protein that is capable of triggering cell-cell fusion in vitro.
FGFRL1是成纤维细胞生长因子受体(FGFR)家族的第五个成员。与其他成员相似,它在细胞外结构域含有三个免疫球蛋白环,但与其他受体不同的是,它缺乏通过转磷酸化进行信号传导所需的细胞内蛋白酪氨酸激酶结构域。FGFRL1主要存在于肌肉骨骼系统中,在那里它似乎抑制细胞增殖,但诱导细胞黏附和分化。FGFRL1基因靶向破坏的小鼠在出生时因膈肌畸形而死亡,这种膈肌在出生后不够强壮,无法使肺部充气。在成肌细胞分化为多核肌管的过程中,FGFRL1的表达高度上调,这表明FGFRL1在细胞-细胞融合中起重要作用。最近我们发现FGFRL1确实能诱导培养细胞融合成大的多核细胞。报告基因检测表明,FGFRL1的第三个免疫球蛋白结构域和跨膜结构域对于将CHO细胞融合成包含数百个细胞核的多核细胞既是必需的也是充分的。在接触部位,融合细胞呈现出一种特殊的网状结构,其孔隙直径约为1微米。这些结构可能代表着具有融合孔的膜区域,这些融合孔启动了细胞-细胞融合过程。FGFRL1是第一种能够在体外触发细胞-细胞融合的哺乳动物蛋白。