Group:MUZIC:MLP

Cystein and glycine-rich protein 3 (CSRP3)

spinqualitylabelsall models The N-terminal LIM domain of MLP (PDB entry [1]) Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image   Print3D Introduction The Cystein and glycine-rich protein 3 (CSRP3), or as also known as Muscle LIM Protein (MLP), is one of the three CSRP family members identified in vertebrates. CSRP3 has been identified muscle and cardiac cells. The three family members contain 192-194 residues and two LIM domains adjacent to a flexible glycine-rich linker. Each LIM domain comprises two Zn-binding motifs CCHC and CCCC representing a structural and presumably functional independent unit. Sequence Annotation CSRP3 comprises 194 aminoacids with two LIM domains followed by glycine reach sequences (CSRP3_HUMAN). Structures LIM domains have been intensively characterized using NMR. The solution structures exist for human MLP and other vertebrate CRPs family members. Function and Interactions All three CRPs are associated with the actin cytoskeleton and have similar functions in different muscle varieties. MLP (or CRP3) is localized in Z and M-lines in striated muscles. The interactions of MLP with α-actinin, telethonin, βI-spectrin, N-RAP (Nebulin-related-anchoring protein) and cofilin2 (CFL2) have been suggested in the literature, underlying the essential role of MLP as a scaffold protein in the sarcomere. During myogenesis MLP has been suggested to form complexes with the muscle helix-loop-helix transcription factors MyoD, MRF4 and myogenin. Pathology Mutations on MLP are reported to be involved in cardiac myopathies. The MLP mutation W4R is disrupting the interaction with [telethonin] and is responsible for Dilated Cardiomyopathy (DCM)

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Nikos Pinotsis