Oliveira Sandra Marisa, Cardoso Isabel, Saraiva Maria João
a Molecular Neurobiology, IBMC- Instituto de Biologia Molecular e Celular, Rua do Campo Alegre, 823, 4150-180, Porto, Portugal.
b Escola Superior de Tecnologia da Saúde do Porto, Instituto Politécnico do Porto, Portugal.
Expert Rev Endocrinol Metab. 2012 Mar;7(2):181-189. doi: 10.1586/eem.12.2.
Transthyretin (TTR) is a plasma- and cerebrospinal fluid-circulating protein. Besides the primordially attributed systemic role as a transporter molecule of thyroxine (T) and retinol (through the binding to retinol-binding protein [RBP]), TTR has been recognized as a protein with important functions in several aspects of the nervous system physiology. TTR has been shown to play an important role in behavior, cognition, amidated neuropeptide processing and nerve regeneration. Furthermore, it has been proposed that TTR is neuroprotective in Alzheimer's disease and cerebral ischemia. Mutations in TTR are a well-known cause of familial amyloidotic polyneuropathy, an autosomal dominant neurodegenerative disorder characterized by systemic deposition of TTR amyloid fibrils, particularly in the peripheral nervous system. The purpose of this review is to highlight the roles of TTR in the nervous system, beyond its systemic role as a transporter molecule of T and RBP-retinol.
转甲状腺素蛋白（TTR）是一种在血浆和脑脊液中循环的蛋白质。除了最初作为甲状腺素（T）和视黄醇（通过与视黄醇结合蛋白[RBP]结合）的转运分子所具有的全身作用外，TTR还被认为是一种在神经系统生理学的多个方面具有重要功能的蛋白质。TTR已被证明在行为、认知、酰胺化神经肽加工和神经再生中发挥重要作用。此外，有人提出TTR在阿尔茨海默病和脑缺血中具有神经保护作用。TTR突变是家族性淀粉样多神经病的一个众所周知的病因，这是一种常染色体显性神经退行性疾病，其特征是TTR淀粉样纤维在全身沉积，尤其是在周围神经系统。本综述的目的是强调TTR在神经系统中的作用，而不仅仅是其作为T和RBP-视黄醇转运分子的全身作用。
