Kv1.1 knock-in ataxic mice exhibit spontaneous myokymic activity exacerbated by fatigue, ischemia and low temperature

scientific article

Kv1.1 knock-in ataxic mice exhibit spontaneous myokymic activity exacerbated by fatigue, ischemia and low temperature is …
instance of (P31):
scholarly articleQ13442814

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P356DOI10.1016/J.NBD.2012.05.002
P932PMC publication ID3402927
P698PubMed publication ID22609489
P5875ResearchGate publication ID225048927

P50authorGiuseppe Di GiovanniQ42469477
P2093author name stringMauro Pessia
Vito Enrico Pettorossi
Marina Mora
Christian Zammit
Fabio Massimo Botti
Maria Cristina D'Adamo
Mario Valentino
Orazio Brunetti
Paola Imbrici
Richard Muscat
Sara Gibertini
P2860cites workCaspr2, a new member of the neurexin superfamily, is localized at the juxtaparanodes of myelinated axons and associates with K+ channelsQ22011028
A common ankyrin-G-based mechanism retains KCNQ and NaV channels at electrically active domains of the axonQ24307090
Episodic ataxia results from voltage-dependent potassium channels with altered functionsQ24312207
A proton-gated cation channel involved in acid-sensingQ24329227
Functional implications of the localization and activity of acid-sensitive channels in rat peripheral nervous systemQ24530236
Episodic ataxia type-1 mutations in the hKv1.1 cytoplasmic pore region alter the gating properties of the channelQ24533155
Myokymia and neonatal epilepsy caused by a mutation in the voltage sensor of the KCNQ2 K+ channelQ24555140
Sodium channel Na(v)1.6 is localized at nodes of ranvier, dendrites, and synapsesQ24653551
Clinical, genetic, and expression studies of mutations in the potassium channel gene KCNA1 reveal new phenotypic variabilityQ28140752
Mutations in the KCNA1 gene associated with episodic ataxia type-1 syndrome impair heteromeric voltage-gated K(+) channel functionQ28140870
A mouse model of episodic ataxia type-1Q28213245
Episodic ataxia mutations in Kv1.1 alter potassium channel function by dominant negative effects or haploinsufficiencyQ28266460
Expanding the phenotype of potassium channelopathy: severe neuromyotonia and skeletal deformities without prominent Episodic AtaxiaQ28280670
A novel KCNA1 mutation associated with global delay and persistent cerebellar dysfunctionQ28308294
Determinants of excitability at transition zones in Kv1.1-deficient myelinated nervesQ78003472
Heteromultimeric K+ channels in terminal and juxtaparanodal regions of neuronsQ28505460
Localization of Kv1.1 and Kv1.2, two K channel proteins, to synaptic terminals, somata, and dendrites in the mouse brainQ28513040
Temperature-sensitive neuromuscular transmission in Kv1.1 null mice: role of potassium channels under the myelin sheath in young nervesQ28584818
Localization and targeting of voltage-dependent ion channels in mammalian central neurons.Q30492681
Activity-dependent regulation of mitochondrial motility by calcium and Na/K-ATPase at nodes of Ranvier of myelinated nervesQ30532624
Calcium imaging in live rat optic nerve myelinated axons in vitro using confocal laser microscopyQ30946388
Analysis of potassium channel functions in mammalian axons by gene knockoutsQ33876736
Nerve excitability studies characterize Kv1.1 fast potassium channel dysfunction in patients with episodic ataxia type 1.Q34372981
Pathways modulating neural KCNQ/M (Kv7) potassium channelsQ34464180
Voltage-dependent calcium block of normal and tetramethrin-modified single sodium channelsQ34536274
Calmodulin mediates Ca2+-dependent modulation of M-type K+ channelsQ36447189
Axonal activation-induced calcium transients in myelinating Schwann cells, sources, and mechanisms.Q36700921
Episodic ataxia type 1: a neuronal potassium channelopathyQ36774725
Matching mitochondria to metabolic needs at nodes of RanvierQ37868834
The C-terminal domain of ßIV-spectrin is crucial for KCNQ2 aggregation and excitability at nodes of Ranvier.Q39641825
Lactic Acidosis RevisitedQ40144977
Cellular mechanisms of muscle fatigue.Q40808559
Mechanisms underlying modulation of neuronal KCNQ2/KCNQ3 potassium channels by extracellular protonsQ41939192
Lactate enhances the acid-sensing Na+ channel on ischemia-sensing neuronsQ43723705
Differential expression of K+ channel mRNAs in the rat brain and down-regulation in the hippocampus following seizuresQ44448404
KCNQ2 is a nodal K+ channel.Q44755361
Extra- and intracellular pH during near-complete forebrain ischemia in the rat.Q48382563
Deletion of the K(V)1.1 potassium channel causes epilepsy in miceQ48472743
Episodic ataxia type 1 mutations affect fast inactivation of K+ channels by a reduction in either subunit surface expression or affinity for inactivation domain.Q48681599
Peripheral nerve hyperexcitability due to dominant-negative KCNQ2 mutationsQ48782947
Familial paroxysmal kinesigenic ataxia and continuous myokymiaQ48886983
Function and distribution of three types of rectifying channel in rat spinal root myelinated axons.Q51795801
Action potentials induce uniform calcium influx in mammalian myelinated optic nerves.Q51937877
Computer model for action potential propagation through branch point in myelinated nerves.Q52069207
Myelinating Schwann cells determine the internodal localization of Kv1.1, Kv1.2, Kvbeta2, and Caspr.Q52538564
Decorin and biglycan expression is differentially altered in several muscular dystrophies.Q52566349
Muscarinic suppression of a novel voltage-sensitive K+ current in a vertebrate neuroneQ59066538
Neurological ChannelopathiesQ60243424
Hereditary myokymia and periodic ataxiaQ67293676
Intracellular calcium directly inhibits potassium M channels in excised membrane patches from rat sympathetic neuronsQ70901383
On the physiological role of internodal potassium channels and the security of conduction in myelinated nerve fibresQ72574408
Evidence for the presence of potassium channels in the paranodal region of acutely demyelinated mammalian single nerve fibresQ72906597
P433issue3
P407language of work or nameEnglishQ1860
P304page(s)310-321
P577publication date2012-05-17
P1433published inNeurobiology of DiseaseQ15716606
P1476titleKv1.1 knock-in ataxic mice exhibit spontaneous myokymic activity exacerbated by fatigue, ischemia and low temperature
P478volume47

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cites work (P2860)
Q33870521A channelopathy mutation in the voltage-sensor discloses contributions of a conserved phenylalanine to gating properties of Kv1.1 channels and ataxia
Q89761539Cisplatin-Induced Skeletal Muscle Dysfunction: Mechanisms and Counteracting Therapeutic Strategies
Q30449041K(+) channelepsy: progress in the neurobiology of potassium channels and epilepsy
Q40443495Kv1.1 channelopathy abolishes presynaptic spike width modulation by subthreshold somatic depolarization
Q33901811Lack of neuropathy-related phenotypes in hint1 knockout mice.
Q38239544Mutational consequences of aberrant ion channels in neurological disorders
Q26782897New insights into the pathogenesis and therapeutics of episodic ataxia type 1
Q34969098Novel phenotype associated with a mutation in the KCNA1(Kv1.1) gene
Q44425941Pharmacological characteristics of Kv1.1- and Kv1.2-containing channels are influenced by the stoichiometry and positioning of their α subunits
Q36500042The episodic ataxia type 1 mutation I262T alters voltage-dependent gating and disrupts protein biosynthesis of human Kv1.1 potassium channels.
Q34390785Voltage-gated potassium channels at the crossroads of neuronal function, ischemic tolerance, and neurodegeneration