2310046A06Rik Activators

The protein known as muscular LMNA-interacting protein refers to a protein that interacts with the LMNA gene product, lamin A/C, within muscle cells. Lamin A/C is a type of intermediate filament protein that provides structural support to the nucleus by forming part of the nuclear lamina, a dense fibrillar network inside the nuclear envelope.Mutations in the LMNA gene can lead to a variety of diseases collectively termed laminopathies, which often include muscle-related conditions such as Emery-Dreifuss muscular dystrophy, limb-girdle muscular dystrophy, and dilated cardiomyopathy with conduction system disease. These conditions can affect skeletal and cardiac muscle, leading to muscle weakness, contractures, and heart problems.

The muscular LMNA-interacting protein likely plays a role in maintaining nuclear integrity, mechano-transduction, and gene regulation within muscle cells by directly interacting with lamin A/C. Through this interaction, it may be involved in the mechanical stabilization of the nucleus, especially during muscle contraction and relaxation cycles. The protein may also be implicated in the organization of the nuclear envelope and chromatin structure, which is crucial for proper gene expression.In the context of laminopathies, the interaction between lamin A/C and the muscular LMNA-interacting protein may become disrupted, which can contribute to the pathogenesis of muscle degeneration and weakness. Understanding the specific functions of this interacting protein and its relationship with lamin A/C is essential for developing targeted therapies for diseases associated with LMNA mutations.