Varaico: Literature-Extracted Genetic Variants

Gene:KCNA1
Variant:p.V408L
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KCNA1

NM_000217.3:p.V408L

Associated Papers

The genetic spectrum features of 2261 Chinese children with epilepsy and intellectual disability.

Pang N et al.

Bmc Medicine, 2025

40598568 (PubMed )
Supplement: 12916_2025_4220_MOESM1_ESM.xlsx › Table_S3

Clinical and Functional Study of a De Novo Variant in the PVP Motif of Kv1.1 Channel Associated with Epilepsy, Developmental Delay and Ataxia.

Dinoi G et al.

International Journal of Molecular Sciences, 2022

35897654 (PubMed )
Supplement: ijms-23-08079-s001.zip › ijms-1792331-supplementary.pdf

Interictal Headache, Pseudodystonia, and Persistent Ataxia in Episodic Ataxia Type 1 Due to a Novel KCNA1 Gene Mutation.

Bhattacharjee S et al.

Movement Disorders Clinical Practice, 2022

Case report: A novel loss-of-function pathogenic variant in the KCNA1 cytoplasmic N-terminus causing carbamazepine-responsive type 1 episodic ataxia.

Manville RW et al.

Frontiers in Neurology, 2022

36016548 (PubMed )
Supplement: Data_Sheet_2.PDF

Therapeutic Potential of Sodium Channel Blockers as a Targeted Therapy Approach in KCNA1-Associated Episodic Ataxia and a Comprehensive Review of the Literature.

Lauxmann S et al.

Frontiers in Neurology, 2021

A Common Kinetic Property of Mutations Linked to Episodic Ataxia Type 1 Studied in the Shaker Kv Channel.

Zhao J et al.

International Journal of Molecular Sciences, 2020

Two novel KCNA1 variants identified in two unrelated Chinese families affected by episodic ataxia type 1 and neurodevelopmental disorders.

Yuan H et al.

Molecular Genetics & Genomic Medicine, 2020

Kv1.1 Channelopathies: Pathophysiological Mechanisms and Therapeutic Approaches.

D'Adamo MC et al.

International Journal of Molecular Sciences, 2020

Clinical Spectrum of KCNA1 Mutations: New Insights into Episodic Ataxia and Epilepsy Comorbidity.

Paulhus K et al.

International Journal of Molecular Sciences, 2020

De novo KCNA1 variants in the PVP motif cause infantile epileptic encephalopathy and cognitive impairment similar to recurrent KCNA2 variants.

Rogers A et al.

American Journal of Medical Genetics. Part A, 2018

Scn2a deletion improves survival and brain-heart dynamics in the Kcna1-null mouse model of sudden unexpected death in epilepsy (SUDEP).

Mishra V et al.

Human Molecular Genetics, 2017

Episodic ataxia type 1: clinical characterization, quality of life and genotype-phenotype correlation.

Graves TD et al.

Brain : a Journal of Neurology, 2014

Mechanism of accelerated current decay caused by an episodic ataxia type-1-associated mutant in a potassium channel pore.

Peters CJ et al.

The Journal of Neuroscience : the Official Journal of the Society for Neuroscience, 2011

[Hereditary episodic ataxia].

Riant F et al.

Revue Neurologique, 2011

A novel KCNA1 mutation associated with global delay and persistent cerebellar dysfunction.

Demos MK et al.

Movement Disorders : Official Journal of the Movement Disorder Society, 2009

Cervical Dystonia with Cerebellar Ataxia in KCNA1 Mutation: A Phenotypic Expansion.

Mahale RR et al.

Annals of Indian Academy of Neurology, 0

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