Protein Information

Name Ca v
Synonyms CA V; CA VA; CA5; CA5A; CAV; CAVA; Carbonate dehydratase VA; Carbonic anhydrase V…

Compound Information

Name 4-aminopyridine
CAS 4-pyridinamine

Reference List

PubMed Abstract RScore(About this table)
16686648 Weiergraber M, Henry M, Krieger A, Kamp M, Radhakrishnan K, Hescheler J, Schneider T: Altered seizure susceptibility in mice lacking the Ca (v) 2.3 E-type Ca2+ channel. Epilepsia. 2006 May;47(5):839-50.

In addition, convulsive seizure activity was induced by systemic administration of either 4-aminopyridine (4-AP; 10 mg/kg, i.p.) or pentylenetetrazol (PTZ; 80 mg/kg, s.c.) to reveal possible alterations in seizure susceptibility.
7(0,0,0,7) Details
17699699 Cataldi M, Lariccia V, Marzaioli V, Cavaccini A, Curia G, Viggiano D, Canzoniero LM, di Renzo G, Avoli M, Annunziato L: Zn (2+) slows down Ca (V) 3.3 gating kinetics: implications for thalamocortical activity. J Neurophysiol. 2007 Oct;98(4):2274-84. Epub 2007 Aug 15.

In line with this finding, we discovered that chelation of endogenous Zn (2+) decreased the frequency of occurrence of ictal-like epileptiform discharges in rat thalamocortical slices perfused with medium containing the convulsant 4-aminopyridine (50 microM).
3(0,0,0,3) Details
17395137 Strupp M, Zwergal A, Brandt T: Episodic ataxia type 2. . Neurotherapeutics. 2007 Apr;4(2):267-73.

EA 2 is caused most often by the loss of function mutations of the calcium channel gene CACNA1A, which encodes the Ca (v) 2.1 subunit of the P/Q-type calcium channel and is primarily expressed in Purkinje cells.
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.
1(0,0,0,1) Details
19302149 Ladera C, Martin R, Bartolome-Martin D, Torres M, Sanchez-Prieto J: Partial compensation for N-type Ca (2+) channel loss by P/Q-type Ca (2+) channels underlines the differential release properties supported by these channels at cerebrocortical nerve terminals. Eur J Neurosci. 2009 Mar;29(6):1131-40.


In addition, we have determined the release properties at terminals from mice lacking the alpha (1B) subunit of N-type channels (Ca (v) 2.2) to test the possibility that P/Q-type channels can compensate for the loss of N-type Ca (2+) channels.
1(0,0,0,1) 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.

Both the human and mouse disorders result from mutations in CACNA1A, the gene encoding the alpha (1) 2.1 subunit of Ca (v) 2.1 voltage-gated calcium channels.
These mutations predict reduced calcium 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.
1(0,0,0,1) Details
19955484 Jiang P, Rushing SN, Kong CW, Fu J, Lieu DK, Chan CW, Deng W, Li RA: Electrophysiological properties of human induced pluripotent stem cells. . Am J Physiol Cell Physiol. 2010 Mar;298(3):C486-95. Epub 2009 Dec 2.


By contrast, 4-aminopyridine (4-AP) inhibited viability (EC (50) = 4.5 +/- 0.5 mM) but had less effect on proliferation (EC (50) = 0.9 +/- 0.5 mM).
0(0,0,0,0) Details
16091557 Wang K, Xue T, Tsang SY, Van Huizen R, Wong CW, Lai KW, Ye Z, Cheng L, Au KW, Zhang J, Li GR, Lau CP, Tse HF, Li RA: Electrophysiological properties of pluripotent human and mouse embryonic stem cells. Stem Cells. 2005 Nov-Dec;23(10):1526-34. Epub 2005 Aug 9.


In mESCs, tetraethylammonium (TEA)-sensitive depolarization-activated delayed rectifier K+ currents (IK (DR)) (8.6 +/- 0.9 pA/pF at +40 mV; IC50 = 1.2 +/- 0.3 mM), which contained components sensitive to 4-aminopyridine (4-AP) (IC50 = 0.5 +/- 0.1 mM) and 100 nM Ca2+-activated K+ current (IK (Ca)) blocker iberiotoxin (IBTX),were detected in 52.3% of undifferentiated cells.IK (DR) was similarly present in hESCs (approximately 100%) but with an approximately sixfold higher current density (47.5 +/- 7.9 pA/pF at +40 mV).
0(0,0,0,0) Details