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- Title
αll Spectrin Forms a Periodic Cytoskeleton at the Axon Initial Segment and Is Required for Nervous System Function.
- Authors
Yu-Mei Huang, Claire; Chuansheng Zhang; Szu-Yu Ho, Tammy; Oses-Prieto, Juan; Burlingame, Alma L.; Lalonde, Joshua; Noebels, Jeffrey L.; Leterrier, Christophe; Rasband, Matthew N.
- Abstract
Spectrins form a submembranous cytoskeleton proposed to confer strength and flexibility to neurons and to participate in ion channel clustering at axon initial segments (AIS) and nodes of Ranvier. Neuronal spectrin cytoskeletons consist of diverse β subunits and αII spectrin. Although αII spectrin is found in neurons in both axonal and somatodendritic domains, using proteomics, biochemistry, and superresolution microscopy, we show that αII and (31V spectrin interact and form a periodic AIS cytoskeleton. To determine the role of spectrins in the nervous system, we generated Sptanl f/f mice for deletion of CNS αII spectrin. We analyzed αII spectrin-deficient mice of both sexes and found that loss of all spectrin causes profound reductions in all β spectrins. αII spectrin-deficient mice die before 1 month of age and have disrupted AIS and many other neurological impairments including seizures, disrupted cortical lamination, and widespread neurodegeneration. These results demonstrate the importance of the spectrin cytoskeleton both at the AIS and throughout the nervous system.
- Subjects
SPECTRIN; CYTOSKELETON; NERVOUS system; ANKYRINS; NODES of Ranvier
- Publication
Journal of Neuroscience, 2017, Vol 37, Issue 47, p11311
- ISSN
0270-6474
- Publication type
Article
- DOI
10.1523/JNEUROSCI.2112-17.2017