Protein Details: Potassium voltage-gated channel subfamily KQT member 2

Protein ID

ICDB_Pro_0217

Protein Name

Potassium voltage-gated channel subfamily KQT member 2

Gene Name

KCNQ2

Organism

Homo sapiens (Human)

Length

872 amino acids

AlphaFoldDB

AF-O43526-F1-model_v4.pdb

Function

Associates with KCNQ3 to form a potassium channel with essentially identical properties to the channel underlying the native M-current;a slowly activating and deactivating potassium conductance which plays a critical role in determining the subthreshold electrical excitability of neurons as well as the responsiveness to synaptic inputs. Therefore;it is important in the regulation of neuronal excitability. KCNQ2/KCNQ3 current is blocked by linopirdine and XE991;and activated by the anticonvulsant retigabine. As the native M-channel;the potassium channel composed of KCNQ2 and KCNQ3 is also suppressed by activation of the muscarinic acetylcholine receptor CHRM1. KCNQ2-KCNQ3 channel is selectively permeable to other cations besides potassium;in decreasing order of affinity K(+) > Rb(+) > Cs(+) > Na(+). Associates with Na(+)-coupled myo-inositol symporter SLC5A3 forming a coregulatory complex that alters ion selectivity;increasing Na(+) and Cs(+) permeation relative to K(+) permeation

Sequence

MVQKSRNGGVYPGPSGEKKLKVGFVGLDPGAPDSTRDGALLIAGSEAPKRGSILSKPRAGGAGAGKPPKRNAFYRKLQNFLYNVLERPRGWAFIYHAYVFLLVFSCLVLSVFSTIKEYEKSSEGALYILEIVTIVVFGVEYFVRIWAAGCCCRYRGWRGRLKFARKPFCVIDIMVLIASIAVLAAGSQGNVFATSALRSLRFLQILRMIRMDRRGGTWKLLGSVVYAHSKELVTAWYIGFLCLILASFLVYLAEKGENDHFDTYADALWWGLITLTTIGYGDKYPQTWNGRLLAATFTLIGVSFFALPAGILGSGFALKVQEQHRQKHFEKRRNPAAGLIQSAWRFYATNLSRTDLHSTWQYYERTVTVPMYSSQTQTYGASRLIPPLNQLELLRNLKSKSGLAFRKDPPPEPSPSKGSPCRGPLCGCCPGRSSQKVSLKDRVFSSPRGVAAKGKGSPQAQTVRRSPSADQSLEDSPSKVPKSWSFGDRSRARQAFRIKGAASRQNSEEASLPGEDIVDDKSCPCEFVTEDLTPGLKVSIRAVCVMRFLVSKRKFKESLRPYDVMDVIEQYSAGHLDMLSRIKSLQSRVDQIVGRGPAITDKDRTKGPAEAELPEDPSMMGRLGKVEKQVLSMEKKLDFLVNIYMQRMGIPPTETEAYFGAKEPEPAPPYHSPEDSREHVDRHGCIVKIVRSSSSTGQKNFSAPPAAPPVQCPPSTSWQPQSHPRQGHGTSPVGDHGSLVRIPPPPAHERSLSAYGGGNRASMEFLRQEDTPGCRPPEGNLRDSDTSISIPSVDHEELERSFSGFSISQSKENLDALNSCYAAVAPCAKVRPYIAEGESDTDSDLCTPCGPPPRSATGEGPFGDVGWAGPRK

PDB Structures

5J03 6FEG 6FEH 7CR0 7CR1 7CR2 7CR3 7CR4 7CR7

Ligand Binding

1. DICL_CP

2. DICL_Pep

Binding Site

Disease

Developmental And Epileptic Encephalopathy 7 and Seizures;Benign Familial Neonatal;1

Location

In adult and fetal brain. Highly expressed in areas containing neuronal cell bodies; low in spinal cord and corpus callosum. Isoform 2 is preferentially expressed in differentiated neurons. Isoform 6 is prominent in fetal brain; undifferentiated neuroblastoma cells and brain tumors.

DOI ID

10.1093/dnares/3.5.311; 10.1038/ng0198-25; 10.1126/science.279.5349.403; 10.1126/science.282.5395.1890; 10.1016/s0014-5793(98)01296-4; 10.1074/jbc.273.31.19419; 10.1523/jneurosci.21-04-01096.2001; 10.1038/414865a; 10.1101/gr.2596504; 10.1038/25367; 10.1523/jneurosci.19-18-07742.1999; 10.1016/s0014-5793(00)01918-9; 10.1074/jbc.275.18.13343; 10.1523/jneurosci.20-05-01710.2000; 10.1111/j.1469-7793.2000.t01-2-00349.x; 10.1124/mol.58.2.253; 10.1124/mol.58.3.591; 10.1016/s0304-3940(00)00866-1; 10.1073/pnas.090092797; 10.1186/gb-2004-5-2-r8; 10.1073/pnas.0509122102; 10.1074/jbc.m116.722637; 10.1016/j.bpj.2017.06.055; 10.1021/acs.biochem.6b00477; 10.1007/pl00008713; 10.1038/sj.ejhg.5200570; 10.1073/pnas.211431298; 10.1093/braiwg286; 10.1016/s0920-1211(03)00037-8; 10.1212/01.wnl.0000132979.08394.6d; 10.1212/01.wnl.0000275523.95103.36; 10.1111/epi.12089; 10.1093/hmg/ddu030; 10.1111/epi.12954; 10.1111/epi.13020; 10.1523/jneurosci.4423-14.2015; 10.1111/cge.12636; 10.1136/jmedgenet-2015-103263; 10.1002/humu.23149

RefSeq

NP_004509.2 [O43526-3]; NP_742104.1 [O43526-2]; NP_742105.1 [O43526-1]; NP_742106.1 [O43526-4]; NP_742107.1 [O43526-6]