cd18419

BTB (Broad-Complex, Tramtrack and Bric a brac)/POZ (poxvirus and zinc finger) domain found in potassium voltage-gated channel subfamily D member 3 (KCND3)

CDD entry
Member databaseCDD
CDD typedomain
Short nameBTB_POZ_KCND3
SetBTB_POZ

Description

KCND3, also called voltage-gated potassium channel subunit Kv4.3, is a pore-forming subunit of voltage-gated rapidly inactivating A-type potassium channels. Mutations in KCND3 cause spinocerebellar ataxia. Voltage-gated potassium (Kv) channels are composed of alpha subunits, which form the actual conductance pore, and cytoplasmic beta subunits, which are auxiliary proteins that associate with alpha subunits to modulate the activity of the Kv channel. KCND3 is an alpha subunit that forms functional homo- or hetero-tetrameric channels (with other Kv4/KCND alpha subunits) through its BTB/POZ domain, also known as tetramerization (T1) domain, which is a versatile protein-protein interaction motif. It is modulated by cytoplasmic KChIPs/KCNIPs (Kv-channel interacting proteins), which are small calcium binding proteins with EF-hand-like domains.
[16, 1, 2, 6, 9, 3, 17, 12, 19, 11, 4, 18, 10, 5, 15, 8, 14, 13, 7]

References

1.Heteropoda toxin 2 interaction with Kv4.3 and Kv4.1 reveals differences in gating modification. DeSimone CV, Zarayskiy VV, Bondarenko VE, Morales MJ. Mol Pharmacol 80, 345-55, (2011). PMID: 21540294

2.Mutations in potassium channel kcnd3 cause spinocerebellar ataxia type 19. Duarri A, Jezierska J, Fokkens M, Meijer M, Schelhaas HJ, den Dunnen WF, van Dijk F, Verschuuren-Bemelmans C, Hageman G, van de Vlies P, Kusters B, van de Warrenburg BP, Kremer B, Wijmenga C, Sinke RJ, Swertz MA, Kampinga HH, Boddeke E, Verbeek DS. Ann Neurol 72, 870-80, (2012). PMID: 23280838

3.Novel mutations in the KCND3-encoded Kv4.3 K+ channel associated with autopsy-negative sudden unexplained death. Giudicessi JR, Ye D, Kritzberger CJ, Nesterenko VV, Tester DJ, Antzelevitch C, Ackerman MJ. Hum Mutat 33, 989-97, (2012). PMID: 22457051

4.Gene structures and expression profiles of three human KCND (Kv4) potassium channels mediating A-type currents I(TO) and I(SA). Isbrandt D, Leicher T, Waldschutz R, Zhu X, Luhmann U, Michel U, Sauter K, Pongs O. Genomics 64, 144-54, (2000). PMID: 10729221

5.Mutations in the genes KCND2 and KCND3 encoding the ion channels Kv4.2 and Kv4.3, conducting the cardiac fast transient outward current (ITO,f), are not a frequent cause of long QT syndrome. Frank-Hansen R, Larsen LA, Andersen P, Jespersgaard C, Christiansen M. Clin Chim Acta 351, 95-100, (2005). PMID: 15563876

6.Mutations in KCND3 cause spinocerebellar ataxia type 22. Lee YC, Durr A, Majczenko K, Huang YH, Liu YC, Lien CC, Tsai PC, Ichikawa Y, Goto J, Monin ML, Li JZ, Chung MY, Mundwiller E, Shakkottai V, Liu TT, Tesson C, Lu YC, Brice A, Tsuji S, Burmeister M, Stevanin G, Soong BW. Ann Neurol 72, 859-69, (2012). PMID: 23280837

7.Effects of neferine on Kv4.3 channels expressed in HEK293 cells and ex vivo electrophysiology of rabbit hearts. Wang C, Chen YF, Quan XQ, Wang H, Zhang R, Xiao JH, Wang JL, Zhang CT, Xiang JZ, Tang Q. Acta Pharmacol Sin 36, 1451-61, (2015). PMID: 26592512

8.β Subunits Control the Effects of Human Kv4.3 Potassium Channel Phosphorylation. Abbott GW. Front Physiol 8, 646, (2017). PMID: 28919864

9.Transient outward current (I(to)) gain-of-function mutations in the KCND3-encoded Kv4.3 potassium channel and Brugada syndrome. Giudicessi JR, Ye D, Tester DJ, Crotti L, Mugione A, Nesterenko VV, Albertson RM, Antzelevitch C, Schwartz PJ, Ackerman MJ. Heart Rhythm 8, 1024-32, (2011). PMID: 21349352

10.Divergent regulation of cardiac KCND3 potassium channel expression by the thyroid hormone receptors alpha1 and beta1. Gassanov N, Er F, Michels G, Zagidullin N, Brandt MC, Hoppe UC. J Physiol 587, 1319-29, (2009). PMID: 19171649

11.Modulation of Kv4.3 current by accessory subunits. Deschenes I, Tomaselli GF. FEBS Lett 528, 183-8, (2002). PMID: 12297301

12.Structural basis for modulation of Kv4 K+ channels by auxiliary KChIP subunits. Wang H, Yan Y, Liu Q, Huang Y, Shen Y, Chen L, Chen Y, Yang Q, Hao Q, Wang K, Chai J. Nat. Neurosci. 10, 32-9, (2007). View articlePMID: 17187064

13.Role of Kv4.3 in Vibration-Induced Muscle Pain in the Rat. Conner LB, Alvarez P, Bogen O, Levine JD. J Pain 17, 444-50, (2016). PMID: 26721612

14.Modulation of human Kv4.3/KChIP2 channel inactivation kinetics by cytoplasmic Ca<sup>2</sup>. Groen C, Bahring R. Pflugers Arch 469, 1457-1470, (2017). PMID: 28735419

15.Genomic organisation and chromosomal localisation of two members of the KCND ion channel family, KCND2 and KCND3. Postma AV, Bezzina CR, de Vries JF, Wilde AA, Moorman AF, Mannens MM. Hum Genet 106, 614-9, (2000). PMID: 10942109

16.Distinct cellular distributions of Kv4 pore-forming and auxiliary subunits in rat dorsal root ganglion neurons. Matsuyoshi H, Takimoto K, Yunoki T, Erickson VL, Tyagi P, Hirao Y, Wanaka A, Yoshimura N. Life Sci 91, 258-63, (2012). PMID: 22820170

17.Kv4 potassium channels form a tripartite complex with the anchoring protein SAP97 and CaMKII in cardiac myocytes. El-Haou S, Balse E, Neyroud N, Dilanian G, Gavillet B, Abriel H, Coulombe A, Jeromin A, Hatem SN. Circ Res 104, 758-69, (2009). PMID: 19213956

18.Cloning and expression of the human kv4.3 potassium channel. Dilks D, Ling HP, Cockett M, Sokol P, Numann R. J. Neurophysiol. 81, 1974-7, (1999). View articlePMID: 10200233

19.Two N-terminal domains of Kv4 K(+) channels regulate binding to and modulation by KChIP1. Scannevin RH, Wang K, Jow F, Megules J, Kopsco DC, Edris W, Carroll KC, Lu Q, Xu W, Xu Z, Katz AH, Olland S, Lin L, Taylor M, Stahl M, Malakian K, Somers W, Mosyak L, Bowlby MR, Chanda P, Rhodes KJ. Neuron 41, 587-98, (2004). View articlePMID: 14980207

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