Na+/ H+ exchanger regulatory factor (NHERF1) is a PDZ domain containing adaptor protein known to bind to various receptors, channels, cytoskeletal elements and cytoplasmic signaling proteins. A 358aa mainly expressed in kidney, liver and pancrease by NHERF gene (chrm 17). And is represented in another isoform, NHERF2. NHERF1 protein contains 2 tandem PDZ domains of approximately 90aa and a C-terminal sequence that binds several members of the ERM (ezrin-radixin-moesin) family of membrane cytoskeletal adapters. The protein plays an important role in regulation of NHE3, turnover of G-protein coupled receptors, platelet derived growth factor receptor and ion transporters such as CFTR, Na/Pi cotransporter, NaHCO3 cotransporter and Trp channels. NHERF1 overexpression in cancers and mutations in NHERF1 targets, Merlin, the product of tumor suppressor gene, also expressed in 2 isoforms, Merlin is involved in pathogenesis of benign tumors of the human nervous system, Merlin is named for its striking similarity with ERM family which thought to link cytoskeletal components with proteins in the cell membrane, It has a binding partner called Syntenin, which is an adapter protein that couples transmembrane proteoglycans to cytoskeletal components.
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