LIM proteins, named for ′LIN11, ISL1, and MEC3,′ are defined by the possession of a highly conserved double zinc finger motif called the LIM domain.[supplied by OMIM
Immunogen
FHL1 (NP_001440, 23 a.a. ~ 120 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
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International journal of medical sciences, 11(3), 262-267 (2014-02-12)
In a subset of patients with Hirschsprung's disease (HSCR), gastrointestinal motor dysfunction persisted long after surgical correction. Gastrointestinal motility is achieved through the coordinated activity of the enteric nervous system, interstitial cells of Cajal, and smooth muscle (SMC) cells. Inhibition
FHL1 is multifunctional and serves as a modular protein binding interface to mediate protein-protein interactions. In skeletal muscle, FHL1 is involved in sarcomere assembly, differentiation, growth, and biomechanical stress. Muscle abnormalities may play a major role in congenital clubfoot (CCF)
Myofibrillar myopathies (MFMs) are histopathologically characterized by desmin-positive protein aggregates and myofibrillar degeneration. While about half of all MFM are caused by mutations in genes encoding sarcomeric and extra-sarcomeric proteins (desmin, filamin C, plectin, VCP, FHL1, ZASP, myotilin, αB-crystallin, and
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