KCNC1 c.959G>A ;(p.R320H)

Variant ID: 11-17793600-G-A

NM_001112741.1(KCNC1):c.959G>A;(p.R320H)

This variant was identified in 27 publications

View GRCh38 version.




Publications:


Network expansion of genetic associations defines a pleiotropy map of human cell biology.

Nature Genetics
Barrio-Hernandez, Inigo I; Schwartzentruber, Jeremy J; Shrivastava, Anjali A; Del-Toro, Noemi N; Gonzalez, Asier A; Zhang, Qian Q; Mountjoy, Edward E; Suveges, Daniel D; Ochoa, David D; Ghoussaini, Maya M; Bradley, Glyn G; Hermjakob, Henning H; Orchard, Sandra S; Dunham, Ian I; Anderson, Carl A CA; Porras, Pablo P; Beltrao, Pedro P
Publication Date: 2023-02-23

Variant appearance in text: KCNC1: 959G>A; Arg320His
PubMed Link: 36823319
Variant Present in the following documents:
  • 41588_2023_1327_MOESM4_ESM.xlsx, sheet 6
View BVdb publication page



Apo and ligand-bound high resolution Cryo-EM structures of the human Kv3.1 channel reveal a novel binding site for positive modulators.

Pnas Nexus
Botte, Mathieu M; Huber, Sophie S; Bucher, Denis D; Klint, Julie K JK; Rodríguez, David D; Tagmose, Lena L; Chami, Mohamed M; Cheng, Robert R; Hennig, Michael M; Abdul Rahman, Wassim W
Publication Date: 2022-07

Variant appearance in text: Kv3.1: R320H
PubMed Link: 36741467
Variant Present in the following documents:
  • Main text
  • pgac083.pdf
  • pgac083_supplemental_file.pdf
View BVdb publication page



A KCNC1-related neurological disorder due to gain of Kv3.1 function.

Annals Of Clinical And Translational Neurology
Clatot, Jerome J; Ginn, Natalie N; Costain, Gregory G; Goldberg, Ethan M EM
Publication Date: 2022-11-23

Variant appearance in text: KCNC1: Arg320His
PubMed Link: 36419348
Variant Present in the following documents:
  • Main text
  • ACN3-10-111.pdf
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Genetic Testing to Inform Epilepsy Treatment Management From an International Study of Clinical Practice.

Jama Neurology
McKnight, Dianalee D; Morales, Ana A; Hatchell, Kathryn E KE; Bristow, Sara L SL; Bonkowsky, Joshua L JL; Perry, Michael Scott MS; Berg, Anne T AT; Borlot, Felippe F; Esplin, Edward D ED; Moretz, Chad C; Angione, Katie K; Ríos-Pohl, Loreto L; Nussbaum, Robert L RL; Aradhya, Swaroop S; , ; Haldeman-Englert, Chad R CR; Levy, Rebecca J RJ; Parachuri, Venu G VG; Lay-Son, Guillermo G; de Montellano, David J Dávila-Ortiz DJD; Ramirez-Garcia, Miguel Angel MA; Benítez Alonso, Edmar O EO; Ziobro, Julie J; Chirita-Emandi, Adela A; Felix, Temis M TM; Kulasa-Luke, Dianne D; Megarbane, Andre A; Karkare, Shefali S; Chagnon, Sarah L SL; Humberson, Jennifer B JB; Assaf, Melissa J MJ; Silva, Sebastian S; Zarroli, Katherine K; Boyarchuk, Oksana O; Nelson, Gary R GR; Palmquist, Rachel R; Hammond, Katherine C KC; Hwang, Sean T ST; Boutlier, Susan B SB; Nolan, Melinda M; Batley, Kaitlin Y KY; Chavda, Devraj D; Reyes-Silva, Carlos Alberto CA; Miroshnikov, Oleksandr O; Zuccarelli, Britton B; Amlie-Wolf, Louise L; Wheless, James W JW; Seinfeld, Syndi S; Kanhangad, Manoj M; Freeman, Jeremy L JL; Monroy-Santoyo, Susana S; Rodriguez-Vazquez, Natalia N; Ryan, Monique M MM; Machie, Michelle M; Guerra, Patricio P; Hassan, Muhammad Jawad MJ; Candee, Meghan S MS; Bupp, Caleb P CP; Park, Kristen L KL; Muller, Eric E; Lupo, Pamela P; Pedersen, Robert C RC; Arain, Amir M AM; Murphy, Andrea A; Schatz, Krista K; Mu, Weiyi W; Kalika, Paige M PM; Plaza, Lautaro L; Kellogg, Marissa A MA; Lora, Evelyn G EG; Carson, Robert P RP; Svystilnyk, Victoria V; Venegas, Viviana V; Luke, Rebecca R RR; Jiang, Huiyuan H; Stetsenko, Tetiana T; Dueñas-Roque, Milagros M MM; Trasmonte, Joseph J; Burke, Rebecca J RJ; Hurst, Anna C E ACE; Smith, Douglas M DM; Massingham, Lauren J LJ; Pisani, Laura L; Costin, Carrie E CE; Ostrander, Betsy B; Filloux, Francis M FM; Ananth, Amitha L AL; Mohamed, Ismail S IS; Nechai, Alla A; Dao, Jasmin M JM; Fahey, Michael C MC; Aliu, Ermal E; Falchek, Stephen S; Press, Craig A CA; Treat, Lauren L; Eschbach, Krista K; Starks, Angela A; Kammeyer, Ryan R; Bear, Joshua J JJ; Jacobson, Mona M; Chernuha, Veronika V; Meibos, Bailey B; Wong, Kristen K; Sweney, Matthew T MT; Espinoza, A Chris AC; Van Orman, Colin B CB; Weinstock, Arie A; Kumar, Ashutosh A; Soler-Alfonso, Claudia C; Nolan, Danielle A DA; Raza, Muhammad M; Rojas Carrion, Miguel David MD; Chari, Geetha G; Marsh, Eric D ED; Shiloh-Malawsky, Yael Y; Parikh, Sumit S; Gonzalez-Giraldo, Ernesto E; Fulton, Stephen S; Sogawa, Yoshimi Y; Burns, Kaitlyn K; Malets, Myroslava M; Montiel Blanco, Johnny David JD; Habela, Christa W CW; Wilson, Carey A CA; Guzmán, Guillermo G GG; Pavliuk, Mariia M
Publication Date: 2022-10-31

Variant appearance in text: KCNC1: 959G>A; Arg320His
PubMed Link: 36315135
Variant Present in the following documents:
  • jamaneurol-e223651-s001.pdf
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Whole-exome analysis of 177 pediatric patients with undiagnosed diseases.

Scientific Reports
Narita, Kotaro K; Muramatsu, Hideki H; Narumi, Satoshi S; Nakamura, Yuji Y; Okuno, Yusuke Y; Suzuki, Kyogo K; Hamada, Motoharu M; Yamaguchi, Naoya N; Suzuki, Atsushi A; Nishio, Yosuke Y; Shiraki, Anna A; Yamamori, Ayako A; Tsumura, Yusuke Y; Sawamura, Fumi F; Kawaguchi, Masahiro M; Wakamatsu, Manabu M; Kataoka, Shinsuke S; Kato, Kohji K; Asada, Hideyuki H; Kubota, Tetsuo T; Muramatsu, Yukako Y; Kidokoro, Hiroyuki H; Natsume, Jun J; Mizuno, Seiji S; Nakata, Tomohiko T; Inagaki, Hidehito H; Ishihara, Naoko N; Yonekawa, Takahiro T; Okumura, Akihisa A; Ogi, Tomoo T; Kojima, Seiji S; Kaname, Tadashi T; Hasegawa, Tomonobu T; Saitoh, Shinji S; Takahashi, Yoshiyuki Y
Publication Date: 2022-08-26

Variant appearance in text: KCNC1: R320H
PubMed Link: 36028527
Variant Present in the following documents:
  • 41598_2022_14161_MOESM2_ESM.xlsx, sheet 1
View BVdb publication page



Timing is everything: structural insights into the disease-linked Kv3 channels controlling fast action-potential firing in the brain.

Nature Communications
Gunthorpe, Martin J MJ
Publication Date: 2022-07-15

Variant appearance in text: Kv3.1: R320H
PubMed Link: 35840565
Variant Present in the following documents:
  • Main text
  • 41467_2022_Article_31537.pdf
View BVdb publication page



Epilepsy Combined With Multiple Gene Heterozygous Mutation.

Frontiers In Pediatrics
Qiuju, He H; Jianlong, Zhuang Z; Qi, Wen W; Zhifa, Li L; Ding, Wang W; Xiaofang, Sun S; Yingjun, Xie X
Publication Date: 2022

Variant appearance in text: KCNC1: 959G>A
PubMed Link: 35299674
Variant Present in the following documents:
  • Main text
  • fped-10-763642.pdf
View BVdb publication page



Progressive Myoclonus Epilepsies: Diagnostic Yield With Next-Generation Sequencing in Previously Unsolved Cases.

Neurology. Genetics
Canafoglia, Laura L; Franceschetti, Silvana S; Gambardella, Antonio A; Striano, Pasquale P; Giallonardo, Anna Teresa AT; Tinuper, Paolo P; Di Bonaventura, Carlo C; Michelucci, Roberto R; Ferlazzo, Edoardo E; Granata, Tiziana T; Magaudda, Adriana A; Licchetta, Laura L; Filla, Alessandro A; La Neve, Angela A; Riguzzi, Patrizia P; Cantisani, Teresa Anna TA; Fanella, Martina M; Castellotti, Barbara B; Gellera, Cinzia C; Bahlo, Melanie M; Zara, Federico F; Courage, Carolina C; Lehesjoki, Anna-Elina AE; Oliver, Karen L KL; Berkovic, Samuel F SF
Publication Date: 2021-12

Variant appearance in text: KCNC1: 959G>A; Arg320His
PubMed Link: 34786481
Variant Present in the following documents:
  • NG2021017194.pdf
View BVdb publication page



Kv3.1 channelopathy: a novel loss-of-function variant and the mechanistic basis of its clinical phenotypes.

Annals Of Translational Medicine
Li, Xiaoyang X; Zheng, Yongsheng Y; Li, Shaoyuan S; Nair, Umesh U; Sun, Chong C; Zhao, Chongbo C; Lu, Jiahong J; Zhang, Victor Wei VW; Maljevic, Snezana S; Petrou, Steven S; Lin, Jie J
Publication Date: 2021-09

Variant appearance in text: Kv3.1: Arg320His
PubMed Link: 34733949
Variant Present in the following documents:
  • Main text
  • atm-09-18-1397.pdf
View BVdb publication page



Gene Editing and Modulation: the Holy Grail for the Genetic Epilepsies?

Neurotherapeutics : The Journal Of The American Society For Experimental Neurotherapeutics
Carpenter, Jenna C JC; Lignani, Gabriele G
Publication Date: 2021-07

Variant appearance in text: KCNC1: 959G>A
PubMed Link: 34235638
Variant Present in the following documents:
  • 13311_2021_Article_1081.pdf
View BVdb publication page



Progressive myoclonus epilepsies-Residual unsolved cases have marked genetic heterogeneity including dolichol-dependent protein glycosylation pathway genes.

American Journal Of Human Genetics
Courage, Carolina C; Oliver, Karen L KL; Park, Eon Joo EJ; Cameron, Jillian M JM; Grabińska, Kariona A KA; Muona, Mikko M; Canafoglia, Laura L; Gambardella, Antonio A; Said, Edith E; Afawi, Zaid Z; Baykan, Betul B; Brandt, Christian C; di Bonaventura, Carlo C; Chew, Hui Bein HB; Criscuolo, Chiara C; Dibbens, Leanne M LM; Castellotti, Barbara B; Riguzzi, Patrizia P; Labate, Angelo A; Filla, Alessandro A; Giallonardo, Anna T AT; Berecki, Geza G; Jackson, Christopher B CB; Joensuu, Tarja T; Damiano, John A JA; Kivity, Sara S; Korczyn, Amos A; Palotie, Aarno A; Striano, Pasquale P; Uccellini, Davide D; Giuliano, Loretta L; Andermann, Eva E; Scheffer, Ingrid E IE; Michelucci, Roberto R; Bahlo, Melanie M; Franceschetti, Silvana S; Sessa, William C WC; Berkovic, Samuel F SF; Lehesjoki, Anna-Elina AE
Publication Date: 2021-04-01

Variant appearance in text: KCNC1: 959G>A; Arg320His
PubMed Link: 33798445
Variant Present in the following documents:
  • Main text
View BVdb publication page



Progressive myoclonus epilepsy KCNC1 variant causes a developmental dendritopathy.

Epilepsia
Carpenter, Jenna C JC; Männikkö, Roope R; Heffner, Catherine C; Heneine, Jana J; Sampedro-Castañeda, Marisol M; Lignani, Gabriele G; Schorge, Stephanie S
Publication Date: 2021-05

Variant appearance in text: KCNC1: 959G>A; R320H
PubMed Link: 33735526
Variant Present in the following documents:
  • Main text
View BVdb publication page



Identification of Core Genes and Screening of Potential Targets in Glioblastoma Multiforme by Integrated Bioinformatic Analysis.

Frontiers In Oncology
Yang, Ji'an J; Yang, Qian Q
Publication Date: 2020

Variant appearance in text: KCNC1: 959G>A; Arg320His
PubMed Link: 33718116
Variant Present in the following documents:
  • Main text
  • fonc-10-615976.pdf
View BVdb publication page



Channelopathies Are a Frequent Cause of Genetic Ataxias Associated with Cerebellar Atrophy.

Movement Disorders Clinical Practice
Gauquelin, Laurence L; Hartley, Taila T; Tarnopolsky, Mark M; Dyment, David A DA; Brais, Bernard B; Geraghty, Michael T MT; Tétreault, Martine M; Ahmed, Sohnee S; Rojas, Samantha S; Choquet, Karine K; Majewski, Jacek J; Bernier, François F; Innes, Allan Micheil AM; Rouleau, Guy G; Suchowersky, Oksana O; Boycott, Kym M KM; Yoon, Grace G
Publication Date: 2020-11

Variant appearance in text: KCNC1: 959G>A; Arg320His
PubMed Link: 33163565
Variant Present in the following documents:
  • Main text
View BVdb publication page



A longitudinal footprint of genetic epilepsies using automated electronic medical record interpretation.

Genetics In Medicine : Official Journal Of The American College Of Medical Genetics
Ganesan, Shiva S; Galer, Peter D PD; Helbig, Katherine L KL; McKeown, Sarah E SE; O'Brien, Margaret M; Gonzalez, Alexander K AK; Felmeister, Alex S AS; Khankhanian, Pouya P; Ellis, Colin A CA; Helbig, Ingo I
Publication Date: 2020-12

Variant appearance in text: KCNC1: Arg320His
PubMed Link: 32773773
Variant Present in the following documents:
  • 41436_2020_923_MOESM3_ESM.xls, sheet 1
View BVdb publication page



Possible precision medicine implications from genetic testing using combined detection of sequence and intragenic copy number variants in a large cohort with childhood epilepsy.

Epilepsia Open
Truty, Rebecca R; Patil, Nila N; Sankar, Raman R; Sullivan, Joseph J; Millichap, John J; Carvill, Gemma G; Entezam, Ali A; Esplin, Edward D ED; Fuller, Amy A; Hogue, Michelle M; Johnson, Britt B; Khouzam, Amirah A; Kobayashi, Yuya Y; Lewis, Rachel R; Nykamp, Keith K; Riethmaier, Darlene D; Westbrook, Jody J; Zeman, Michelle M; Nussbaum, Robert L RL; Aradhya, Swaroop S
Publication Date: 2019-09

Variant appearance in text: KCNC1: 959G>A; Arg320His
PubMed Link: 31440721
Variant Present in the following documents:
  • EPI4-4-397-s003.xlsx, sheet 1
View BVdb publication page



Encephalopathies with KCNC1 variants: genotype-phenotype-functional correlations.

Annals Of Clinical And Translational Neurology
Cameron, Jillian M JM; Maljevic, Snezana S; Nair, Umesh U; Aung, Ye Htet YH; Cogné, Benjamin B; Bézieau, Stéphane S; Blair, Edward E; Isidor, Bertrand B; Zweier, Christiane C; Reis, André A; Koenig, Mary Kay MK; Maarup, Timothy T; Sarco, Dean D; Afenjar, Alexandra A; Huq, A H M Mahbubul AHMM; Kukolich, Mary M; Billette de Villemeur, Thierry T; Nava, Caroline C; Héron, Bénédicte B; Petrou, Steven S; Berkovic, Samuel F SF
Publication Date: 2019-07

Variant appearance in text: KCNC1: Arg320His
PubMed Link: 31353855
Variant Present in the following documents:
  • Main text
  • ACN3-6-1263.pdf
View BVdb publication page



Myoclonus, Epilepsy, and Ataxia Resulting From Potassium Channel Gene Mutation: Expanding the Spectrum Underlying Ramsay Hunt Syndrome.

Movement Disorders Clinical Practice
Balint, Bettina B; Bhatia, Kailash P KP
Publication Date: 2015-09

Variant appearance in text: KCNC1: 959G>A
PubMed Link: 30363511
Variant Present in the following documents:
  • Main text
View BVdb publication page



A leaky voltage sensor domain of cardiac sodium channels causes arrhythmias associated with dilated cardiomyopathy.

Scientific Reports
Moreau, Adrien A; Gosselin-Badaroudine, Pascal P; Mercier, Aurélie A; Burger, Bettina B; Keller, Dagmar I DI; Chahine, Mohamed M
Publication Date: 2018-09-14

Variant appearance in text: Kv3.1: R320H
PubMed Link: 30218094
Variant Present in the following documents:
  • 41598_2018_31772_MOESM1_ESM.pdf
View BVdb publication page



Kv3 Channels: Enablers of Rapid Firing, Neurotransmitter Release, and Neuronal Endurance.

Physiological Reviews
Kaczmarek, Leonard K LK; Zhang, Yalan Y
Publication Date: 2017-10-01

Variant appearance in text: Kv3.1: R320H
PubMed Link: 28904001
Variant Present in the following documents:
  • Main text
View BVdb publication page



Ion Channel Genes and Epilepsy: Functional Alteration, Pathogenic Potential, and Mechanism of Epilepsy.

Neuroscience Bulletin
Wei, Feng F; Yan, Li-Min LM; Su, Tao T; He, Na N; Lin, Zhi-Jian ZJ; Wang, Jie J; Shi, Yi-Wu YW; Yi, Yong-Hong YH; Liao, Wei-Ping WP
Publication Date: 2017-08

Variant appearance in text: KCNC1: R320H
PubMed Link: 28488083
Variant Present in the following documents:
  • Main text
View BVdb publication page



Loss of Function of KCNC1 is associated with intellectual disability without seizures.

European Journal Of Human Genetics : Ejhg
Poirier, Karine K; Viot, Géraldine G; Lombardi, Laura L; Jauny, Clémence C; Billuart, Pierre P; Bienvenu, Thierry T
Publication Date: 2017-05

Variant appearance in text: Kv3.1: 959G>A
PubMed Link: 28145425
Variant Present in the following documents:
  • Main text
View BVdb publication page



iFish: predicting the pathogenicity of human nonsynonymous variants using gene-specific/family-specific attributes and classifiers.

Scientific Reports
Wang, Meng M; Wei, Liping L
Publication Date: 2016-08-16

Variant appearance in text: KCNC1: R320H
PubMed Link: 27527004
Variant Present in the following documents:
  • srep31321-s3.xls, sheet 1
View BVdb publication page



Advancing epilepsy genetics in the genomic era.

Genome Medicine
Myers, Candace T CT; Mefford, Heather C HC
Publication Date: 2015-08-25

Variant appearance in text: KCNC1: Arg320His
PubMed Link: 26302787
Variant Present in the following documents:
  • Main text
  • 13073_2015_Article_214.pdf
View BVdb publication page



Molecular pathophysiology and pharmacology of the voltage-sensing module of neuronal ion channels.

Frontiers In Cellular Neuroscience
Miceli, Francesco F; Soldovieri, Maria Virginia MV; Ambrosino, Paolo P; De Maria, Michela M; Manocchio, Laura L; Medoro, Alessandro A; Taglialatela, Maurizio M
Publication Date: 2015

Variant appearance in text: KCNC1: R320H
PubMed Link: 26236192
Variant Present in the following documents:
  • Main text
View BVdb publication page



A recurrent de novo mutation in KCNC1 causes progressive myoclonus epilepsy.

Nature Genetics
Muona, Mikko M; Berkovic, Samuel F SF; Dibbens, Leanne M LM; Oliver, Karen L KL; Maljevic, Snezana S; Bayly, Marta A MA; Joensuu, Tarja T; Canafoglia, Laura L; Franceschetti, Silvana S; Michelucci, Roberto R; Markkinen, Salla S; Heron, Sarah E SE; Hildebrand, Michael S MS; Andermann, Eva E; Andermann, Frederick F; Gambardella, Antonio A; Tinuper, Paolo P; Licchetta, Laura L; Scheffer, Ingrid E IE; Criscuolo, Chiara C; Filla, Alessandro A; Ferlazzo, Edoardo E; Ahmad, Jamil J; Ahmad, Adeel A; Baykan, Betul B; Said, Edith E; Topcu, Meral M; Riguzzi, Patrizia P; King, Mary D MD; Ozkara, Cigdem C; Andrade, Danielle M DM; Engelsen, Bernt A BA; Crespel, Arielle A; Lindenau, Matthias M; Lohmann, Ebba E; Saletti, Veronica V; Massano, João J; Privitera, Michael M; Espay, Alberto J AJ; Kauffmann, Birgit B; Duchowny, Michael M; Møller, Rikke S RS; Straussberg, Rachel R; Afawi, Zaid Z; Ben-Zeev, Bruria B; Samocha, Kaitlin E KE; Daly, Mark J MJ; Petrou, Steven S; Lerche, Holger H; Palotie, Aarno A; Lehesjoki, Anna-Elina AE
Publication Date: 2015-01

Variant appearance in text: KCNC1: 959G>A; Arg320His
PubMed Link: 25401298
Variant Present in the following documents:
  • Main text
View BVdb publication page



Age-related mutations associated with clonal hematopoietic expansion and malignancies.

Nature Medicine
Xie, Mingchao M; Lu, Charles C; Wang, Jiayin J; McLellan, Michael D MD; Johnson, Kimberly J KJ; Wendl, Michael C MC; McMichael, Joshua F JF; Schmidt, Heather K HK; Yellapantula, Venkata V; Miller, Christopher A CA; Ozenberger, Bradley A BA; Welch, John S JS; Link, Daniel C DC; Walter, Matthew J MJ; Mardis, Elaine R ER; Dipersio, John F JF; Chen, Feng F; Wilson, Richard K RK; Ley, Timothy J TJ; Ding, Li L
Publication Date: 2014-12

Variant appearance in text: KCNC1: R320H
PubMed Link: 25326804
Variant Present in the following documents:
  • NIHMS630249-supplement-6.xlsx, sheet 1
  • NIHMS630249-supplement-5.xlsx, sheet 1
View BVdb publication page