IndraLab

"The KCNQ1 gene, encodes the Kv7.1 channel protein, which can form heteromultimers with two other potassium channel proteins, KCNE1 and KCNE3. xref In the human heart the KCNQ1 encodes the pore-forming α subunit, and KCNE1 (also known as minK) encodes the regulatory β subunit of the KCNQ1- KCNE1 complex responsible for I Ks , the slowly activating delayed rectifier K + repolarizing current. xref Mutations in KCNQ1 are associated with hereditary LQTS1 (also known as Romano-Ward syndrome), Jervell and Lange-Nielsen syndrome, and familial atrial fibrillation. xref In 2002, Marx et al showed that modulation of I KS β-adrenergic receptor stimulation requires targeting of cyclic adenosine 3′,5′-monophosphate (cAMP)–dependent PKA and protein phosphatase 1 (PP1) to hKCNQ1 through the A kinase-anchoring protein (AKAP)-9, also known as yotiao. xref These authors elegantly demonstrated that yotiao binds to the human KCNQ1 by a leucine zipper motif, which is disrupted by an LQTS mutation (hKCNQ1-G589D)."