Редактирование: KCNC4

Potassium voltage-gated channel subfamily C member 4 (KSHIIIC) (Voltage-gated potassium channel subunit Kv3.4) [C1orf30]

==Publications==

{{medline-entry
|title=Targeted deletion of [i]Kcne3[/i] impairs skeletal muscle function in mice.
|pubmed-url=https://pubmed.ncbi.nlm.nih.gov/28356343
|abstract=[[KCNE3]] (MiRP2) forms heteromeric voltage-gated K  channels with the skeletal muscle-expressed [[KCNC4]] (K 3.4) α subunit. [i][[KCNE3]][/i] was the first reported skeletal muscle K  channel disease gene, but the requirement for [i][[KCNE3]][/i] in skeletal muscle has been questioned. Here, we confirmed [[KCNE3]] transcript and protein expression in mouse skeletal muscle using [i]Kcne3[/i]  tissue as a negative control. Whole-transcript microarray analysis (770,317 probes, interrogating 28,853 transcripts) findings were consistent with [i]Kcne3[/i] deletion increasing gastrocnemius oxidative metabolic gene expression and the proportion of type IIa fast-twitch oxidative muscle fibers, which was verified using immunofluorescence. The down-regulated transcript set overlapped with muscle unloading gene expression profiles (≥1.5-fold change; [i]P[/i] < 0.05). Gastrocnemius K  channel α subunit remodeling arising from [i]Kcne3[/i] deletion was highly specific, involving just 3 of 69 α subunit genes probed: known [[KCNE3]] partners [[KCNC4]] and [[KCNH2]] (mERG) were down-regulated, and [[KCNK4]] (TRAAK) was up-regulated ([i]P[/i] < 0.05). Functionally, [i]Kcne3[/i]  mice exhibited abnormal hind-limb clasping upon tail suspension (63% of [i]Kcne3[/i]  mice ≥10-mo-old [i]vs.[/i] 0% age-matched [i]Kcne3[/i]  littermates). Whereas 5 of 5 [i]Kcne3[/i]  mice exhibited the typical biphasic decline in contractile force with repetitive stimuli of hind-limb muscle, both [i]in vivo[/i] and [i]in vitro,[/i] this was absent in 6 of 6 [i]Kcne3[/i]  mice tested. Finally, myoblasts isolated from [i]Kcne3[/i]  mice exhibit faster-inactivating and smaller sustained outward currents than those from [i]Kcne3[/i]  mice. Thus, [i]Kcne3[/i] deletion impairs skeletal muscle function in mice.-King, E. C., Patel, V., Anand, M., Zhao, X., Crump, S. M., Hu, Z., Weisleder, N., Abbott, G. W. Targeted deletion of [i]Kcne3[/i] impairs skeletal muscle function in mice.
|mesh-terms=* Aging
* Animals
* Down-Regulation
* Female
* Gene Expression Regulation
* Mice
* Mice, Knockout
* Muscle Contraction
* Muscle, Skeletal
* Myoblasts
* Potassium Channels, Voltage-Gated
* Protein Array Analysis
* Transcriptome
* Up-Regulation
|keywords=* Kv3.4
* MiRP2
* myotonia
* periodic paralysis
* potassium channel
|full-text-url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5472403
}}