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ササキ タカヒロ
Sasaki Takahiro
佐々木 孝寛 所属 医学部 医学科（東京女子医科大学病院） 職種 助教 |
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| 論文種別 | 原著 |
| 言語種別 | 英語 |
| 査読の有無 | 査読あり |
| 表題 | Peripherin is a subunit of peripheral nerve neurofilaments: implications for differential vulnerability of CNS and peripheral nervous system axons. |
| 掲載誌名 | 正式名：The Journal of neuroscience : the official journal of the Society for Neuroscience 略 称：J Neurosci ISSNコード：15292401/02706474 |
| 掲載区分 | 国外 |
| 巻・号・頁 | 32(25),pp.8501-8 |
| 国際共著 | 国際共著 |
| 著者・共著者 | Yuan Aidong, Sasaki Takahiro, Kumar Asok, Peterhoff Corrinne M, Rao Mala V, Liem Ronald K, Julien Jean-Pierre, Nixon Ralph A |
| 担当区分 | 2nd著者 |
| 発行年月 | 2012/06 |
| 概要 | Peripherin, a neuronal intermediate filament protein implicated in neurodegenerative disease, coexists with the neurofilament triplet proteins [neurofilament light (NFL), medium (NFM), and heavy (NFH) chain] but has an unknown function. The earlier peak expression of peripherin than the triplet during brain development and its ability to form homopolymers, unlike the triplet, which are obligate heteropolymers, have supported a widely held view that peripherin and neurofilament triplets form separate filament systems. However, here, we demonstrate that, despite a postnatal decline in expression, peripherin is as abundant as the triplet in the adult PNS and exists in a relatively fixed stoichiometry with these subunits. Peripherin exhibits a distribution pattern identical to those of triplet proteins in sciatic axons and colocalizes with NFL on single neurofilaments by immunogold electron microscopy. Peripherin also coassembles into a single network of filaments containing NFL, NFM, and NFH with and without α-internexin in quadruple- or quintuple-transfected SW13vim(-) cells. Genetically deleting NFL in mice dramatically reduces peripherin content in sciatic axons. Moreover, peripherin mutations has been shown to disrupt the neurofilament network in transfected SW13vim(-) cells. These data show that peripherin and the neurofilament proteins are functionally interdependent. The results strongly support the view that, rather than forming an independent structure, peripherin is a subunit of neurofilaments in the adult PNS. Our findings provide a basis for its close relationship with neurofilaments in PNS diseases associated with neurofilament accumulation. |
| DOI | 10.1523/JNEUROSCI.1081-12.2012 |
| PMID | 22723690 |