In agreement with the association of Nesprin1alpha with photoreceptor ciliary rootlets and the functional interaction between rootletin and Nesprin1 in fibroblasts, the authors demonstrate that multiple isoforms of Nesprin1 are integral components of ciliary rootlets of multiciliated ependymal and tracheal cells. | Multiple Isoforms of Nesprin1 Are Integral Components of Ciliary Rootlets. Potter C, Zhu W, Razafsky D, Ruzycki P, Kolesnikov AV, Doggett T, Kefalov VJ, Betleja E, Mahjoub MR, Hodzic D., Free PMC Article | 08/4/2018 |
Nesprin-1alpha recruits Akap450 to the nuclear envelope (NE)independently of kinesin and that Akap450, but not other centrosomal proteins, is required for microtubule nucleation from the NE. | Nesprin-1α-Dependent Microtubule Nucleation from the Nuclear Envelope via Akap450 Is Necessary for Nuclear Positioning in Muscle Cells. Gimpel P, Lee YL, Sobota RM, Calvi A, Koullourou V, Patel R, Mamchaoui K, Nédélec F, Shackleton S, Schmoranzer J, Burke B, Cadot B, Gomes ER., Free PMC Article | 08/4/2018 |
Nesprin 1alpha2, which lacks actin-binding domains, was crucial for postnatal viability, nuclear positioning, and skeletal muscle function. | Nesprin 1α2 is essential for mouse postnatal viability and nuclear positioning in skeletal muscle. Stroud MJ, Feng W, Zhang J, Veevers J, Fang X, Gerace L, Chen J., Free PMC Article | 09/16/2017 |
the positioning of nuclei requires PCM-1, a protein of the centrosome that relocalizes to the nuclear envelope at the onset of differentiation in a manner that is dependent on the presence of nesprin-1. | Nuclear alignment in myotubes requires centrosome proteins recruited by nesprin-1. Espigat-Georger A, Dyachuk V, Chemin C, Emorine L, Merdes A. | 08/5/2017 |
Results suggest that the loss of function of KASH-LESS variant of Nesprin1 giant resulting from Nesprin1 nonsense mutations underlies the molecular etiology of autosomal recessive cerebellar ataxia Type I. | A variant of Nesprin1 giant devoid of KASH domain underlies the molecular etiology of autosomal recessive cerebellar ataxia type I. Razafsky D, Hodzic D., Free PMC Article | 04/2/2016 |
Disruption of both nesprin 1 and desmin results in decreased lifespan, body weight and muscle strength. | Disruption of both nesprin 1 and desmin results in nuclear anchorage defects and fibrosis in skeletal muscle. Chapman MA, Zhang J, Banerjee I, Guo LT, Zhang Z, Shelton GD, Ouyang K, Lieber RL, Chen J., Free PMC Article | 07/25/2015 |
nesprin-dependent recruitment of kinesin-1 to the nuclear envelope through the interaction of a conserved LEWD motif with kinesin light chain might be a general mechanism for cell-type-specific nuclear positioning during development. | Nesprins anchor kinesin-1 motors to the nucleus to drive nuclear distribution in muscle cells. Wilson MH, Holzbaur EL., Free PMC Article | 02/21/2015 |
a role for Nesprin-1 in the DNA damage response pathway | Nesprin-1 role in DNA damage response. Sur I, Neumann S, Noegel AA., Free PMC Article | 01/24/2015 |
Loss of Nesprin 1 or 2, or both, led to impairment of gene expression changes in response to biomechanical stimuli. These data suggest a model whereby biomechanical signals are communicated from proteins of the outer nuclear membrane | Targeted ablation of nesprin 1 and nesprin 2 from murine myocardium results in cardiomyopathy, altered nuclear morphology and inhibition of the biomechanical gene response. Banerjee I, Zhang J, Moore-Morris T, Pfeiffer E, Buchholz KS, Liu A, Ouyang K, Stroud MJ, Gerace L, Evans SM, McCulloch A, Chen J., Free PMC Article | 12/20/2014 |
Nesprin-1 played important roles in mouse embryonic stem cell differentiation into cardiomyocytes. | [Effect of nesprin-1 in the differentiation of mouse embryonic stem cells into cardiomyocytes]. Gao F, Yi QJ, Tian YJ, Wang FJ, Liu GX. | 06/1/2013 |
Data show that low expression of Syne1 splicing isoforms accounts for the wider and irregular nuclear envelope inter-membrane space in embryonic stem cells. | Increased expression of Syne1/nesprin-1 facilitates nuclear envelope structure changes in embryonic stem cell differentiation. Smith ER, Zhang XY, Capo-Chichi CD, Chen X, Xu XX., Free PMC Article | 01/28/2012 |
Nesprins, but not sun proteins, switch isoforms at the nuclear envelope during muscle development | Nesprins, but not sun proteins, switch isoforms at the nuclear envelope during muscle development. Randles KN, Lam le T, Sewry CA, Puckelwartz M, Furling D, Wehnert M, McNally EM, Morris GE., Free PMC Article | 12/11/2010 |
analysis of nsprin-1 mutations in human and murine cardiomyopathy | Nesprin-1 mutations in human and murine cardiomyopathy. Puckelwartz MJ, Kessler EJ, Kim G, Dewitt MM, Zhang Y, Earley JU, Depreux FF, Holaska J, Mewborn SK, Pytel P, McNally EM., Free PMC Article | 11/27/2010 |
Nesprin 1 is essential for normal positioning and anchorage of nuclei in skeletal muscle. | Nesprin 1 is critical for nuclear positioning and anchorage. Zhang J, Felder A, Liu Y, Guo LT, Lange S, Dalton ND, Gu Y, Peterson KL, Mizisin AP, Shelton GD, Lieber RL, Chen J., Free PMC Article | 03/22/2010 |
The SUN-domain proteins SUN1 and SUN2 and the KASH-domain proteins Syne-1/Nesprin-1 and Syne-2/Nesprin-2 play critical roles in neurogenesis and neuronal migration in mice | SUN1/2 and Syne/Nesprin-1/2 complexes connect centrosome to the nucleus during neurogenesis and neuronal migration in mice. Zhang X, Lei K, Yuan X, Wu X, Zhuang Y, Xu T, Xu R, Han M., Free PMC Article | 01/21/2010 |
Nesprin-1 plays role in neuromuscular disease and Emery Dreifuss muscular dystrophy. | Disruption of nesprin-1 produces an Emery Dreifuss muscular dystrophy-like phenotype in mice. Puckelwartz MJ, Kessler E, Zhang Y, Hodzic D, Randles KN, Morris G, Earley JU, Hadhazy M, Holaska JM, Mewborn SK, Pytel P, McNally EM., Free PMC Article | 01/21/2010 |
TorsinA also associated with the KASH domains of nesprin-1 and -2 | TorsinA binds the KASH domain of nesprins and participates in linkage between nuclear envelope and cytoskeleton. Nery FC, Zeng J, Niland BP, Hewett J, Farley J, Irimia D, Li Y, Wiche G, Sonnenberg A, Breakefield XO., Free PMC Article | 01/21/2010 |
Muscle nuclei failed to aggregate at the NMJ in transgenic mice, demonstrating that localization and positioning of synaptic nuclei require Syne proteins. | Syne proteins anchor muscle nuclei at the neuromuscular junction. Grady RM, Starr DA, Ackerman GL, Sanes JR, Han M., Free PMC Article | 01/21/2010 |
The Nesprin-1 is essential for the interaction with a C-terminal region in SUN1 protein. | The inner nuclear membrane protein Sun1 mediates the anchorage of Nesprin-2 to the nuclear envelope. Padmakumar VC, Libotte T, Lu W, Zaim H, Abraham S, Noegel AA, Gotzmann J, Foisner R, Karakesisoglou I. | 01/21/2010 |
The KASH-domain-containing proteins Syne-1 and Syne-2 play crucial roles in anchoring both synaptic and non-synaptic myonuclei that are important for proper motor neuron innervation and respiration. | Syne-1 and Syne-2 play crucial roles in myonuclear anchorage and motor neuron innervation. Zhang X, Xu R, Zhu B, Yang X, Ding X, Duan S, Xu T, Zhuang Y, Han M. | 01/21/2010 |
SUN1 interacts with nuclear lamin A and cytoplasmic nesprins (nesprin-1 and nesprin-2)to provide a physical connection between the nuclear lamina and the cytoskeleton. | SUN1 interacts with nuclear lamin A and cytoplasmic nesprins to provide a physical connection between the nuclear lamina and the cytoskeleton. Haque F, Lloyd DJ, Smallwood DT, Dent CL, Shanahan CM, Fry AM, Trembath RC, Shackleton S., Free PMC Article | 01/21/2010 |