title: IRBIT: a regulator of ion channels and ion transporters.
abstract: IRBIT (also called AHCYL1) was originally identified as a binding protein of the intracellular Ca(2+) channel inositol 1,4,5-trisphosphate (IP3) receptor and functions as an inhibitory regulator of this receptor. Unexpectedly, many functions have subsequently been identified for IRBIT including the activation of multiple ion channels and ion transporters, such as the Na(+)/HCO3(-) co-transporter NBCe1-B, the Na(+)/H(+) exchanger NHE3, the Cl(-) channel cystic fibrosis transmembrane conductance regulator (CFTR), and the Cl(-)/HCO3(-) exchanger Slc26a6. The characteristic serine-rich region in IRBIT plays a critical role in the functions of this protein. In this review, we describe the evolution, domain structure, expression pattern, and physiological roles of IRBIT and discuss the potential molecular mechanisms underlying the coordinated regulation of these diverse ion channels/transporters through IRBIT. This article is part of a Special Issue entitled: Calcium signaling in health and disease. Guest Editors: Geert Bultynck, Jacques Haiech, Claus W. Heizmann, Joachim Krebs, and Marc Moreau.
authors: Laboratories for Developmental Neurobiology, RIKEN Brain Science Institute, Wako, Saitama 351-0198, Japan.
authors: Hideaki
authors: H
authors: Ando
authors: Laboratories for Developmental Neurobiology, RIKEN Brain Science Institute, Wako, Saitama 351-0198, Japan.
authors: Katsuhiro
authors: K
authors: Kawaai
authors: Laboratories for Developmental Neurobiology, RIKEN Brain Science Institute, Wako, Saitama 351-0198, Japan. Electronic address: mikosiba@brain.riken.jp.
authors: Katsuhiko
authors: K
authors: Mikoshiba
mesh_terms: D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics / Q000378:metabolism*; D005057:Eukaryotic Cells / Q000166:cytology / Q000378:metabolism; D019143:Evolution, Molecular; D005786:Gene Expression Regulation; D006801:Humans; D053496:Inositol 1,4,5-Trisphosphate Receptors / Q000235:genetics / Q000378:metabolism*; D015640:Ion Channel Gating; D037181:Lectins, C-Type / Q000235:genetics / Q000378:metabolism*; D008565:Membrane Proteins / Q000235:genetics / Q000378:metabolism*; D026901:Membrane Transport Proteins / Q000235:genetics / Q000378:metabolism*; D017433:Protein Structure, Secondary; D017434:Protein Structure, Tertiary; D015398:Signal Transduction; D027982:Sodium-Bicarbonate Symporters / Q000235:genetics / Q000378:metabolism*; D000074083:Sodium-Hydrogen Exchanger 3; D017923:Sodium-Hydrogen Exchangers / Q000235:genetics / Q000378:metabolism*; D000076988:Sulfate Transporters
chemical_list: C505032:CFTR protein, human; C470184:CLECL1 protein, human; D053496:Inositol 1,4,5-Trisphosphate Receptors; D037181:Lectins, C-Type; D008565:Membrane Proteins; D026901:Membrane Transport Proteins; C439529:SLC26A6 protein, human; C498898:SLC4A4 protein, human; C000613385:SLC9A3 protein, human; D027982:Sodium-Bicarbonate Symporters; D000074083:Sodium-Hydrogen Exchanger 3; D017923:Sodium-Hydrogen Exchangers; D000076988:Sulfate Transporters; D019005:Cystic Fibrosis Transmembrane Conductance Regulator
keywords: Cl(−) channel, CFTR; Cl(−)/HCO(3)(−) exchanger, Slc26a6; IP3 receptor; IRBIT; Na(+)/H(+) exchanger, NHE3; Na(+)/HCO(3)(−) co-transporter, NBCe1-B