Voltage-gated potassium channel KCNC1

UniProt P48547 Organism Homo sapiens Gene KCNC1

Also known as: KCNC1

Function

Voltage-gated potassium channel that opens in response to the voltage difference across the membrane and through which potassium ions pass in accordance with their electrochemical gradient. The mechanism is time-dependent and inactivation is slow (By similarity). Plays an important role in the rapid repolarization of fast-firing brain neurons (By similarity). Can form functional homotetrameric channels and heterotetrameric channels that contain variable proportions of KCNC2, and possibly other family members as well (By similarity). Contributes to fire sustained trains of very brief action potentials at high frequency in pallidal neurons (By similarity).

Classification

Family (Pfam): PF02214 BTB_2, PF00520 Ion_trans
InterPro: BTB/POZ_dom, Ion_trans_dom, K_chnl_volt-dep_Kv, K_chnl_volt-dep_Kv3, K_chnl_volt-dep_Kv3.1, SKP1/BTB/POZ_sf, T1-type_BTB, VG_K_chnl, Volt_channel_dom_sf
Functional cluster: Nucleic Acid Metabolism & Repair Enzymes

Experimental structures · PDB · 10

7PHH EM 3.20A
7PHI EM 3.10A
7PHK EM 3.10A
7PHL EM 3.20A
7PQT EM 2.65A
7PQU EM 3.03A
8F1C EM 2.92A
8F1D EM 2.94A
8QUC EM 2.90A
8QUD EM 2.50A

A predicted model is available from AlphaFold.

Gene Ontology · 33

Disease associations

progressive myoclonic epilepsy type 7 MONDO:0014521

Drugs targeting this protein · 6

Related proteins · sequence + function similarity

Co-cited proteins · studied together in the literature

Voltage-gated potassium channel KCNC3 1 shared papers
Voltage-gated potassium channel KCNC1 1 shared papers
TBC1 domain family member 24 1 shared papers

Literature · 7 cited papers

Cryo-EM structure of the human Kv3.1 channel reveals gating control by the cytoplasmic T1 domain. Nat. Commun. · 2022
A recurrent de novo mutation in KCNC1 causes progressive myoclonus epilepsy. Nat. Genet. · 2015
Spinocerebellar ataxia-13 Kv3.3 potassium channels: arginine-to-histidine mutations affect both functional and protein expression on the cell surface. Biochem. J. · 2013
Human chromosome 11 DNA sequence and analysis including novel gene identification. Nature · 2006
The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. · 2004
Localization of a highly conserved human potassium channel gene (NGK2-KV4; KCNC1) to chromosome 11p15. Genomics · 1993
The Shaw-related potassium channel gene, Kv3.1, on human chromosome 11, encodes the type l K+ channel in T cells. J. Biol. Chem. · 1992

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