Protein Details: Potassium voltage-gated channel subfamily KQT member 3

Protein ID

ICDB_Pro_0216

Protein Name

Potassium voltage-gated channel subfamily KQT member 3

Gene Name

KCNQ3

Organism

Homo sapiens (Human)

Length

872 amino acids

AlphaFoldDB

AF-O43525-F1-model_v4.pdb

Function

Associates with KCNQ2 or KCNQ5 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 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

MGLKARRAAGAAGGGGDGGGGGGGAANPAGGDAAAAGDEERKVGLAPGDVEQVTLALGAGADKDGTLLLEGGGRDEGQRRTPQGIGLLAKTPLSRPVKRNNAKYRRIQTLIYDALERPRGWALLYHALVFLIVLGCLILAVLTTFKEYETVSGDWLLLLETFAIFIFGAEFALRIWAAGCCCRYKGWRGRLKFARKPLCMLDIFVLIASVPVVAVGNQGNVLATSLRSLRFLQILRMLRMDRRGGTWKLLGSAICAHSKELITAWYIGFLTLILSSFLVYLVEKDVPEVDAQGEEMKEEFETYADALWWGLITLATIGYGDKTPKTWEGRLIAATFSLIGVSFFALPAGILGSGLALKVQEQHRQKHFEKRRKPAAELIQAAWRYYATNPNRIDLVATWRFYESVVSFPFFRKEQLEAASSQKLGLLDRVRLSNPRGSNTKGKLFTPLNVDAIEESPSKEPKPVGLNNKERFRTAFRMKAYAFWQSSEDAGTGDPMAEDRGYGNDFPIEDMIPTLKAAIRAVRILQFRLYKKKFKETLRPYDVKDVIEQYSAGHLDMLSRIKYLQTRIDMIFTPGPPSTPKHKKSQKGSAFTFPSQQSPRNEPYVARPSTSEIEDQSMMGKFVKVERQVQDMGKKLDFLVDMHMQHMERLQVQVTEYYPTKGTSSPAEAEKKEDNRYSDLKTIICNYSETGPPEPPYSFHQVTIDKVSPYGFFAHDPVNLPRGGPSSGKVQATPPSSATTYVERPTVLPILTLLDSRVSCHSQADLQGPYSDRISPRQRRSITRDSDTPLSLMSVNHEELERSPSGFSISQDRDDYVFGPNGGSSWMREKRYLAEGETDTDTDPFTPSGSMPLSSTGDGISDSVWTPSNKPI

PDB Structures

5J03

Ligand Binding

1. DICL_CP

2. DICL_Pep

Binding Site

Disease

Seizures;Benign Familial Neonatal;2 and Benign Familial Neonatal Epilepsy

Location

Predominantly expressed in brain.

DOI ID

10.1038/25367; 10.1074/jbc.273.31.19419; 10.1038/ng1285; 10.1038/nature04406; 10.1101/gr.2596504; 10.1038/ng0198-53; 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; 10.1038/sj.bjp.0703861; 10.1073/pnas.0509122102; 10.1074/jbc.m116.722637; 10.1016/j.bpj.2017.06.055; 10.1021/acs.biochem.6b00477; 10.1002/1531-8249(200006)47:6<822::aid-ana19>3.3.co;2-o; 10.1093/braiwg286; 10.1111/j.1528-1167.2012.03516.x; 10.1111/epi.12089; 10.1111/epi.13020

RefSeq

NP_001191753.1 [O43525-2]; NP_004510.1 [O43525-1]