• Therefore, we hypothesized that the truncated Kv1.1 would be expressed and dysregulate other Kv1 subunits in the mceph/mceph mice. (biomedcentral.com)
  • Interactions between MCEPH and other Kv1 subunits were studied in cell culture, Xenopus oocytes and the brain. (biomedcentral.com)
  • The channels are hetero- or homotetramers, formed by a great variety of subunits, classified in 12 subfamilies [ 5 ]. (biomedcentral.com)
  • Kv1.1, Kv1.2 and Kv1.4 are the most abundant Kv1 subunits expressed in the brain [ 6 ]. (biomedcentral.com)
  • The N-terminus of the channel is associated with beta subunits that can modify the inactivation properties of the channel as well as affect expression levels. (thermofisher.com)
  • These subunits regulate channel inactivation as well as its expression. (wikipedia.org)
  • brush resides in a cluster of redundant CNGCs encoding subunits which resemble metazoan voltage-gated potassium (Kv1-Kv4) channels in assembly and gating properties. (elifesciences.org)
  • The results reveal a mechanism by which quantitative competition between channel subunits for tetramer assembly can impact the phenotype of the mutation carrier. (elifesciences.org)
  • The responsible mutation was recently revealed to be an eleven base pair deletion, leading to a frame shift, in the gene encoding the potassium channel Kv1.1. (biomedcentral.com)
  • Kv1.1 belongs to the Kv1 subfamily, consisting of eight members (Kv1.1 to Kv1.8). (biomedcentral.com)
  • Western blot analysis of KV1.1 (KCNA1) in Lane 1: rat brain tissue lysate (40 µg), Lane 2: rat testis tissue lysate (40 µg), Lane 3: rat cardiac muscle tissue lysate (40 µg), Lane 4: HeLa whole cell lysate (40 µg), Lane 5: U87 whole cell lysate (40 µg), Lane 6: SHG whole cell lysate (40 µg), Lane 7: NEURO whole cell lysate (40 µg). (thermofisher.com)
  • A synthetic peptide corresponding to a sequence at the C-terminus of human Kv1.1 potassium channel (465-481aa IAHYRQVNIRTANCTTA). (thermofisher.com)
  • Potassium voltage-gated channel subfamily A member 1 also known as Kv1.1 is a shaker related voltage-gated potassium channel that in humans is encoded by the KCNA1 gene. (wikipedia.org)
  • Isaacs syndrome is a result of an autoimmune reaction against the Kv1.1 ion channel. (wikipedia.org)
  • The recommended name for this protein is potassium voltage-gated channel subfamily A member 1 but a number of alternatives have been used in the literature including HuK1 (human K+ channel I), RBK1 (rubidium potassium channel 1), MBK (mouse brain K+ channel), voltage gated potassium channel HBK1, voltage gated potassium channel subunit Kv1.1, voltage-gated K+ channel HuKI and AEMK (associated with myokymia with periodic ataxia). (wikipedia.org)
  • This may affect the channels role in the process of fast inactivation. (wikipedia.org)
  • The C-terminus of the channel is complexed to a PDZ domain protein that is responsible for channel targeting. (thermofisher.com)
  • The protein is believed to have six domains (S1-S6) with the loop between S5 and S6 forming the channel pore. (wikipedia.org)
  • The C-terminus is associated with a PDZ domain protein involved in channel targeting. (wikipedia.org)
  • The protein functions as a potassium selective channel through which the potassium ion may pass in consensus with the electrochemical gradient. (wikipedia.org)
  • The flow of potassium ions is interrupted by interaction of an inactivating particle, an auxiliary protein in humans but an intrinsic part of the channel in other species. (wikipedia.org)
  • Further experiments found that the channel formed by the mutated CNGC protein is leaky and allows calcium ions to enter the cell in the absence of any cue from the environment. (elifesciences.org)
  • These findings provide important insights into the function of CNGCs and reveal how a small change in a channel protein can have far reaching effects on an organism. (elifesciences.org)
  • This gene encodes a voltage-gated delayed potassium channel that is phylogenetically related to the Drosophila Shaker channel. (thermofisher.com)
  • A direct link between potassium channel dysfunction and apoptosis is that reduced intracellular potassium levels appear to promote critical events early in the suicide program. (biomedcentral.com)
  • Cyclic nucleotide-gated channel (CNGC) family members mediate Ca 2+ influx from cellular stores in plants (Charpentier et al. (elifesciences.org)