Protein Domain : Nesprin-1-like, second calponin homology domain IPR047291

Type	Domain
Description	This entry represents the second calponin homology (CH) domain of SYNE-1 and similar proteins predominantly found in vertebrates and arthropodes. CH domains are actin filament (F-actin) binding motifs.Nesprin-1, also called Synaptic nuclear envelope protein 1 (SYNE-1), is a multi-isomeric modular protein which forms a linking network between organelles and the actin cytoskeleton to maintain subcellular spatial organization. SYNE-1 also acts as a component of the LINC (LInker of Nucleoskeleton and Cytoskeleton) complex, which is involved in the connection between the nuclear lamina and the cytoskeleton. This protein is involved in the pathogenesis of Emery Dreifuss muscular dystrophy and lead to a type of autosomal recessive cerebellar ataxia. SYNE-1 contains two copies of the CH domain [ , , , , , , , ]. The homologue from Drosophila melanogaster, named Muscle-specific protein 300 kDa, is also included in this group. It collaborates with Klar to promote even spacing of the myonuclei at the periphery of striated muscle fibres and is essential for anchoring nuclei, mitochondria and endoplasmic reticulum (ER) structures to the Z-disks [, ].
Short Name	CH_SYNE1_rpt2