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首页> 外文期刊>Journal of cellular physiology. >Molecular structure and function of microfibrillar-associated proteins in skeletal and metabolic disorders and cancers
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Molecular structure and function of microfibrillar-associated proteins in skeletal and metabolic disorders and cancers

机译：分子的结构和功能microfibrillar-associated蛋白质骨架代谢紊乱和癌症

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摘要

Microfibrillar-associated proteins (MFAPs) are extracellular matrix glycoproteins, which play a role in microfibril assembly, elastinogenesis, and tissue homeostasis. MFAPs consist of five subfamily members, including MFAP1, MFAP2, MFAP3, MFAP4, and MFAP5. Among these, MFAP2 and MFAP5 are most closely related, and exhibit very limited amino acid sequence homology with MFAP1, MFAP3, and MFAP4. Gene expression profiling analysis reveals that MFAP2, MFAP5, and MFAP4 are specifically expressed in osteoblastic like cells, whereas MFAP1 and MFAP3 are more ubiquitously expressed, indicative of their diverse role in the tropism of tissues. Molecular structural analysis shows that each MFAP family member has distinct features, and functional evidence reveals discrete purposes of individual MFAPs. Animal studies indicate that MFAP2-deficient mice exhibit progressive osteopenia with elevated receptor activator of NF-kappaB ligand (RANKL) expression, whereas MFAP5-deficient mice are neutropenic, and MFAP4-deficient mice displayed emphysema-like pathology and the impaired formation of neointimal hyperplasia. Emerging data also suggest that MFAPs are involved in cancer progression and fat metabolism. Further understanding of tissue-specific pathophy-siology of MFAPs might offer potential novel therapeutic targets for related diseases, such as skeletal and metabolic disorders, and cancers.
机译：Microfibrillar-associated蛋白质(MFAPs)细胞外基质糖蛋白,它发挥作用在微纤维组装、elastinogenesis和组织内稳态。亚科成员,包括MFAP1 MFAP2 MFAP3,MFAP4, MFAP5。最密切相关,表现出很么有限和MFAP1氨基酸序列的同源性,MFAP3, MFAP4。分析表明,MFAP2、MFAP5 MFAP4具体表现在成骨细胞的细胞,而MFAP1和MFAP3更广泛表达,表明他们的不同取向的组织作用。结构分析表明,每个MFAP家庭成员有不同的特性和功能证据显示离散的目的MFAPs。MFAP2-deficient老鼠表现出进步与受体激活升高骨量减少NF-kappaB配体(RANKL)表达,而MFAP5-deficient小鼠粒细胞减少性,MFAP4-deficient老鼠显示emphysema-like病理学和受损的形成血管内膜增生。表明MFAPs参与癌症进展和脂肪代谢。对组织的理解pathophy-siology小说MFAPs可以提供潜在的治疗目标相关的疾病,如骨骼和代谢紊乱,癌症。

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