Progranulin functions as a neurotrophic factor to regulate neurite outgrowth and enhance neuronal survival.
Progranulin functions as a neurotrophic factor to regulate neurite outgrowth and enhance neuronal survival.
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DOI：
10.1083/jcb.200712039
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发表时间：
2008-04-07
期刊：
影响因子：
--
通讯作者：
Robberecht W
中科院分区：
文献类型：
--
作者：
Van Damme P;Van Hoecke A;Lambrechts D;Vanacker P;Bogaert E;van Swieten J;Carmeliet P;Van Den Bosch L;Robberecht W
最近，在前颗粒蛋白（PGRN）基因突变被发现导致家族性和明显散发性额颞叶痴呆（FTLD）。此外，在运动神经元变性患者中发现了PGRN的错义变化，这是一种与FTLD相关的疾病。到目前为止，在FTLD患者中发现的大多数突变都是无效突变。然而，仍然不清楚PGRN蛋白水平是否在这些患者的中枢神经系统中降低。PGRN对神经元的影响也有待确定。我们报道携带PGRN突变的FTLD患者脑脊液中PGRN水平降低。我们观察到，PGRN和GRN E（PGRN的蛋白水解片段之一）促进神经元的存活，并增强培养的神经元中的轴突生长。这些结果表明，PGRN/GRN是一种神经营养因子，其活性可能参与神经系统的发育和神经变性。
Recently, mutations in the progranulin (PGRN) gene were found to cause familial and apparently sporadic frontotemporal lobe dementia (FTLD). Moreover, missense changes in PGRN were identified in patients with motor neuron degeneration, a condition that is related to FTLD. Most mutations identified in patients with FTLD until now have been null mutations. However, it remains unknown whether PGRN protein levels are reduced in the central nervous system from such patients. The effects of PGRN on neurons also remain to be established. We report that PGRN levels are reduced in the cerebrospinal fluid from FTLD patients carrying a PGRN mutation. We observe that PGRN and GRN E (one of the proteolytic fragments of PGRN) promote neuronal survival and enhance neurite outgrowth in cultured neurons. These results demonstrate that PGRN/GRN is a neurotrophic factor with activities that may be involved in the development of the nervous system and in neurodegeneration.
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影响因子：
64.8
作者：
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通讯作者：
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作者：
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