The granulin gene family: from cancer to dementia
The granulin gene family: from cancer to dementia
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DOI：
10.1002/bies.200900086
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发表时间：
2009-11-01
期刊：
影响因子：
4
通讯作者：
Bennett, Hugh P. J.
中科院分区：
文献类型：
--
作者：
Bateman, Andrew;Bennett, Hugh P. J.
生长因子原颗粒(PGRN)调节细胞分裂、存活和迁移。PGRN是一种胞外糖蛋白，含有多个富含半胱氨酸的颗粒蛋白基序。与植物和黏菌中的PGRN家族成员一样，它代表了现代动物中仍然存在的最古老的细胞外调节蛋白之一。PRGN具有多种生物学作用。它有助于调节早期胚胎发育，促进成人组织修复和炎症。PGRN水平升高通常发生在癌症中，而PGRN免疫治疗抑制了小鼠肝癌移植瘤的生长。最近的研究证明了PGRN在神经生物学中的作用。PGRN基因GRN中的常染色体显性突变会导致额叶和颞叶的神经元萎缩，导致额颞叶痴呆。在这篇综述中，我们将讨论目前对PGRN多方面生物学的认识。
The growth factor progranulin (PGRN) regulates cell division, survival, and migration. PGRN is an extracellular glycoprotein bearing multiple copies of the cysteine-rich granulin motif. With PGRN family members in plants and slime mold, it represents one of the most ancient of the extracellular regulatory proteins still extant in modern animals. PRGN has multiple biological roles. It contributes to the regulation of early embryogenesis, to adult tissue repair and inflammation. Elevated PGRN levels often occur in cancers, and PGRN immunotherapy inhibits the growth of hepatic cancer xenografts in mice. Recent studies have demonstrated roles for PGRN in neurobiology. An autosomal dominant mutation in GRN, the gene for PGRN, leads to neuronal atrophy in the frontal and temporal lobes, resulting in the disease frontotemporal lobar dementia. In this review we will discuss current knowledge of the multifaceted biology of PGRN.