| Name | episodic ataxia type 2 |
|---|---|
| Synonyms | APCA; BI; Brain calcium channel 1; Brain calcium channel I; CACH 4; CACH4; CACN 3; CACN3… |
| Name | 4-aminopyridine |
|---|---|
| CAS | 4-pyridinamine |
| PubMed | Abstract | RScore(About this table) | |
|---|---|---|---|
| 18718350 | Strupp M, Kalla R, Glasauer S, Wagner J, Hufner K, Jahn K, Brandt T: Aminopyridines for the treatment of cerebellar and ocular motor disorders. Prog Brain Res. 2008;171:535-41. Animal experiments have shown that aminopyridines [3,4-diaminopyridine (3,4-DAP) and 4-aminopyridine (4-AP)], nonselective blockers of the Kv family of voltage-gated potassium channels, increase Purkinje-cell (PC) excitability. On the basis of these assumptions, we evaluated the efficacy and underlying mechanisms of aminopyridines in DBN and UBN as well as in another cerebellar disorder with an impaired PC function: episodic ataxia type 2 (EA2), which is caused by mutations of the PQ-calcium channel. |
5(0,0,0,5) | Details |
| 15900025 | Strupp M, Kalla R, Freilinger T, Dichgans M, Brandt T: Dysfunction of the brain calcium channel CaV2.1 in absence epilepsy and episodic ataxia--a comment. Brain. 2005 Jun;128(Pt 6):E32; author reply E33. |
1(0,0,0,1) | Details |
| 15136697 | Strupp M, Kalla R, Dichgans M, Freilinger T, Glasauer S, Brandt T: Treatment of episodic ataxia type 2 with the potassium channel blocker 4-aminopyridine. Neurology. 2004 May 11;62(9):1623-5. The authors report three patients with EA2 (two with proven mutations in the CACNA1A gene) whose attacks were prevented with the potassium channel blocker 4-aminopyridine (4-AP; 5 mg tid). |
88(1,1,2,3) | Details |
| 15843617 | Weisz CJ, Raike RS, Soria-Jasso LE, Hess EJ: Potassium channel blockers inhibit the triggers of attacks in the calcium channel mouse mutant tottering. J Neurosci. 2005 Apr 20;25(16):4141-5. These mutations predict reduced currents, particularly in cerebellar Purkinje cells, where these channels are most abundant. 4-Aminopyridine (4-AP), a nonselective blocker of K (v) voltage-gated potassium channels, alleviates attacks of ataxia in EA2 patients. |
83(1,1,1,3) | Details |
| 17395137 | Strupp M, Zwergal A, Brandt T: Episodic ataxia type 2. . Neurotherapeutics. 2007 Apr;4(2):267-73. Two effective treatment options have been established for EA 2: acetazolamide (ACTZ), which probably changes the intracellular pH and thereby the transmembraneous potential, and 4-aminopyridine (4-AP), a potassium channel blocker. |
3(0,0,0,3) | Details |
| 18442126 | Lohle M, Schrempf W, Wolz M, Reichmann H, Storch A: Potassium channel blocker 4-aminopyridine is effective in interictal cerebellar symptoms in episodic ataxia type 2--a video case report. Mov Disord. 2008 Jul 15;23(9):1314-6. |
9(0,0,1,4) | Details |