AMIFAMPRIDINE

Details

Stereochemistry	ACHIRAL
Molecular Formula	C5H7N3
Molecular Weight	109.1292
Optical Activity	NONE
Defined Stereocenters	0 / 0
E/Z Centers	0
Charge	0

SHOW SMILES / InChI

Structure Search

SMILES

NC1=CC=NC=C1N

InChI

InChIKey=OYTKINVCDFNREN-UHFFFAOYSA-N

InChI=1S/C5H7N3/c6-4-1-2-8-3-5(4)7/h1-3H,7H2,(H2,6,8)

HIDE SMILES / InChI

Molecular Formula	C5H7N3
Molecular Weight	109.1292
Charge	0
Count

Stereochemistry	ACHIRAL
Additional Stereochemistry	No
Defined Stereocenters	0 / 0
E/Z Centers	0
Optical Activity	NONE

Description
Sources: http://www.biomarin.com/products/firdapse/ | https://ec.europa.eu/health/documents/community-register/2011/20110412101013/anx_101013_en.pdf | http://www.catalystpharma.com/amifampridine-phosphate.shtml

Amifampridine (Firdapse), currently approved in the European Union, is the first and only approved drug for the symptomatic treatment of Lambert-Eaton Myasthenic Syndrome (LEMS) in adults, a rare autoimmune disease with the primary symptoms of muscle weakness. In LEMS, the body’s own immune system attacks connections between nerves and muscles and disrupts the ability of nerve cells to send signals to muscle cells. Amifampridine blocks voltage-dependent potassium channels, thereby prolonging pre-synaptic cell membrane depolarization. Prolonging the action potential enhances the transport of calcium into the nerve ending. The resulting increase in intracellular calcium concentrations facilitates exocytosis of acetylcholine containing vesicles, which in turn enhances neuromuscular transmission. Amifampridine phosphate has been granted Orphan Drug Designation and Breakthrough Therapy designation by the FDA for the treatment of Lambert-Eaton Myasthenic Syndrome (LEMS).

CNS Activity

Originator

Approval Year

Targets

Primary Target	Pharmacology	Condition	Potency
voltage-gated potassium channel activity 2 Target ID: GO:0005249 Sources: https://ec.europa.eu/health/documents/community-register/2011/20110412101013/anx_101013_en.pdf \| https://www.ncbi.nlm.nih.gov/pubmed/8358554	Blocker 537		2.0 µM [IC50]

Conditions

Condition	Modality	Targets	Highest Phase	Product
Lambert-Eaton myasthenic syndrome 2 Sources: https://ec.europa.eu/health/documents/community-register/2011/20110412101013/anx_101013_en.pdf	Palliative		Approved 8429	Firdapse Approved Use Symptomatic treatment of Lambert-Eaton myasthenic syndrome (LEMS) in adults. Launch Date 2009

C_max

Value	Dose	Analyte	Population
59.1 ng/mL EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/26101174/	20 mg single, oral dose: 20 mg route of administration: Oral experiment type: SINGLE co-administered:	AMIFAMPRIDINE plasma	Homo sapiens population: HEALTHY age: ADULT sex: FEMALE / MALE food status: FASTED
40.6 ng/mL EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/26101174/	20 mg single, oral dose: 20 mg route of administration: Oral experiment type: SINGLE co-administered:	AMIFAMPRIDINE plasma	Homo sapiens population: HEALTHY age: ADULT sex: FEMALE / MALE food status: FED
189 ng/mL EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/26101174/	20 mg single, oral dose: 20 mg route of administration: Oral experiment type: SINGLE co-administered:	3-N-ACETYL-AMIFAMPRIDINE plasma	Homo sapiens population: HEALTHY age: ADULT sex: FEMALE / MALE food status: FED
268 ng/mL EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/26101174/	20 mg single, oral dose: 20 mg route of administration: Oral experiment type: SINGLE co-administered:	3-N-ACETYL-AMIFAMPRIDINE plasma	Homo sapiens population: HEALTHY age: ADULT sex: FEMALE / MALE food status: FASTED

AUC

Value	Dose	Analyte	Population
117 ng × h/mL EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/26101174/	20 mg single, oral dose: 20 mg route of administration: Oral experiment type: SINGLE co-administered:	AMIFAMPRIDINE plasma	Homo sapiens population: HEALTHY age: ADULT sex: FEMALE / MALE food status: FASTED
109 ng × h/mL EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/26101174/	20 mg single, oral dose: 20 mg route of administration: Oral experiment type: SINGLE co-administered:	AMIFAMPRIDINE plasma	Homo sapiens population: HEALTHY age: ADULT sex: FEMALE / MALE food status: FED
1220 ng × h/mL EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/26101174/	20 mg single, oral dose: 20 mg route of administration: Oral experiment type: SINGLE co-administered:	3-N-ACETYL-AMIFAMPRIDINE plasma	Homo sapiens population: HEALTHY age: ADULT sex: FEMALE / MALE food status: FED
1444 ng × h/mL EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/26101174/	20 mg single, oral dose: 20 mg route of administration: Oral experiment type: SINGLE co-administered:	3-N-ACETYL-AMIFAMPRIDINE plasma	Homo sapiens population: HEALTHY age: ADULT sex: FEMALE / MALE food status: FASTED

T_1/2

Value	Dose	Analyte	Population
2.5 h EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/26101174/	20 mg single, oral dose: 20 mg route of administration: Oral experiment type: SINGLE co-administered:	AMIFAMPRIDINE plasma	Homo sapiens population: HEALTHY age: ADULT sex: FEMALE / MALE food status: FASTED
2.28 h EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/26101174/	20 mg single, oral dose: 20 mg route of administration: Oral experiment type: SINGLE co-administered:	AMIFAMPRIDINE plasma	Homo sapiens population: HEALTHY age: ADULT sex: FEMALE / MALE food status: FED
4.03 h EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/26101174/	20 mg single, oral dose: 20 mg route of administration: Oral experiment type: SINGLE co-administered:	3-N-ACETYL-AMIFAMPRIDINE plasma	Homo sapiens population: HEALTHY age: ADULT sex: FEMALE / MALE food status: FED
4.1 h EXPERIMENT https://pubmed.ncbi.nlm.nih.gov/26101174/	20 mg single, oral dose: 20 mg route of administration: Oral experiment type: SINGLE co-administered:	3-N-ACETYL-AMIFAMPRIDINE plasma	Homo sapiens population: HEALTHY age: ADULT sex: FEMALE / MALE food status: FASTED

Doses

Dose	Population	Adverse events
100 mg 1 times / day multiple, oral Highest studied dose Dose: 100 mg, 1 times / day Route: oral Route: multiple Dose: 100 mg, 1 times / day Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2019/209321Orig1s000MedR.pdf	unhealthy, adult Health Status: unhealthy Age Group: adult Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2019/209321Orig1s000MedR.pdf	Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2019/209321Orig1s000MedR.pdf
10 mg 3 times / day multiple, oral Recommended Dose: 10 mg, 3 times / day Route: oral Route: multiple Dose: 10 mg, 3 times / day Sources: https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/209321s000lbl.pdf	healthy, adult n = 52 Health Status: healthy Age Group: adult Population Size: 52 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/209321s000lbl.pdf	Other AEs: Paresthesia, Dysesthesia... Other AEs: Paresthesia (69%) Dysesthesia (69%) Oral dysesthesia (69%) Abdominal pain (25%) Upper abdominal pain (25%) Dyspepsia (17%) Dizziness (12%) Nausea (10%) Back pain (8%) Hypoesthesia (6%) Muscle spasms (6%) Sources: https://www.accessdata.fda.gov/drugsatfda_docs/label/2019/209321s000lbl.pdf
75 mg 1 times / day multiple, oral (typical) Studied dose Dose: 75 mg, 1 times / day Route: oral Route: multiple Dose: 75 mg, 1 times / day Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2019/209321Orig1s000MedR.pdf	unhealthy, adult n = 162 Health Status: unhealthy Age Group: adult Sex: M+F Population Size: 162 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2019/209321Orig1s000MedR.pdf	Other AEs: Convulsion, Head titubation... Other AEs: Convulsion (0.6%) Head titubation (0.6%) Tremor (0.6%) Unresponsive to stimuli (0.6%) Small cell lung cancer (0.6%) Metastases to central nervous system (0.6%) Lung cancer metastatic (0.6%) Pulmonary mass (0.6%) Bradycardia (0.6%) Pneumonia (0.6%) Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2019/209321Orig1s000MedR.pdf

AEs

Overview

CYP3A4	CYP2C9	CYP2D6	hERG
inhibitor 1587 inducer 781	inhibitor 1767	inhibitor 1852	inhibitor 1612

OverviewOther

Other Inhibitor	Other Substrate	Other Inducer
Kv1.3 17 Kv1.5 26 P-gp 1461 OATP1B3 706 CYP1A2 1524 CYP2A6 707 CYP2B6 810 CYP2C8 911 CYP2C19 1478 CYP2E1 882	P-gp 1537 CYP450 65 FMO 35	CYP1A2 701 CYP2B6 547

Drug as perpetrator

Target	Modality	Activity	Metabolite
OATP1B3 715	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no [Inhibition 30 uM]
CYP1A2 1692	inducer 1573 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no	3-N-acetyl-BMN125 2
CYP1A2 1692	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no
CYP2A6 739	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no
CYP2B6 1001	inducer 1573 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no	3-N-acetyl-BMN125 2
CYP2B6 1001	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no
CYP2C19 1553	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no
CYP2C8 965	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no
CYP2C9 1819	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no
CYP2D6 1891	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no
CYP2E1 970	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no
CYP3A4 1925	inducer 1573 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no	3-N-acetyl-BMN125 2
CYP3A4 1925	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no
Kv1.3 32	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=25 Page: 25.0	no	3-N-acetyl-BMN125 2
Kv1.5 41	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=25 Page: 25.0	no	3-N-acetyl-BMN125 2
P-gp 1650	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no
Kv1.5 41	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=25 Page: 25.0	yes [IC50 490.9 uM]
Kv1.3 32	inhibitor 3277 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=25 Page: 25.0	yes [IC50 524.8 uM]
CYP1A2 1692	inducer 1573 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	yes
CYP2B6 1001	inducer 1573 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	yes
CYP3A4 1925	inducer 1573 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	yes

Drug as victim

Target	Modality	Activity
CYP450 65	substrate 3367 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no
FMO 35	substrate 3367 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no
P-gp 1537	substrate 3367 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no

Tox targets

Target	Modality	Activity	Metabolite	Clinical evidence
hERG 1647	inhibitor 1626 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=26 Page: 26.0
hERG 1647	inhibitor 1626 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=26 Page: 26.0		3-N-acetyl-BMN125 2

Sourcing

Vendor/Aggregator	ID	URL
001 Chemical	NO22778	https://www.001chemical.com/chem/54-96-6
3B Scientific (Wuhan) Corp	3B3-000017	http://www.3bsc.com/index/pro_info.php?id=21905
AA Blocks	AA003ICP	http://www.aablocks.com/goods/Goods/detail?id=AA003ICP
AEchem Scientific Corp., USA	AE1-018071	http://www.aechemsc.com/info_products/AE1-018071.php
AHH Chemical co.,ltd	MT-07842	http://www.ahhchemical.com/product/MT-07842.html
AK Scientific, Inc. (AKSCI)	L421	http://www.aksci.com/item_detail.php?cat=L421
Aaron Chemicals	AR003J4H	http://www.aaronchem.com/54-96-6
AbMole Bioscience	M5406	http://www.abmole.com/products/amifampridine.html
Acadechem	ACDX-013489	http://acadechemical.com/product/88181
Acadechem	ACDS-033271	http://www.acadechemical.com/product/107970
Aceschem	ACS010096	https://www.aceschem.com/catalog/54-96-6.html
Achemica	ACMC-209lkh	http://www.achemica.com/prodinfo/?id=128901
Achemo Scientific Limited	AC-11438	http://www.achemo.com/search/?Keyword= 54-96-6
Achemtek	0104-046130	http://www.achemtek.com/54-96-6
Acros Organics	AC112390050	https://www.fishersci.com/shop/products/3-4-diaminopyridine-98-acros-organics-1/AC112390050
Acros Organics	AC112390250	https://www.fishersci.com/shop/products/3-4-diaminopyridine-98-acros-organics/AC112390250
Alfa Aesar	A12465	https://www.alfa.com/en/catalog/A12465/
Alinda	ALBB-005969	http://alinda.ru/finechem_en_.html?i=ALBB-005969
Allbio Pharm Co., Ltd	AF09092	http://www.allbiopharm.com/product/n/AF09092
Ampyridine	AM11000	https://www.ampyridine.com/pyridine/54-96-6.html
Anward	ANW-32223	http://www.anward.com/pro_result/13214
Ark Pharm	AK-26114	http://www.arkpharminc.com/products/54-96-6.html
Aromalake	3,4-Diaminopyridine	http://www.aromalake.com/34-Diaminopyridine-p42.html
Aromalake	LS00018	http://www.aromalake.com/34-Diaminopyridine-p42.html
AstaTech, Inc.	CL0116	http://www.astatechinc.com/CPSResult.php?CRNO=11308
Aurora Fine Chemicals LLC	A00.299.131	http://online.aurorafinechemicals.com/info?ID=A00.299.131
Aurora Fine Chemicals LLC	K01.795.664	http://online.aurorafinechemicals.com/info?ID=K01.795.664
Aurum Pharmatech LLC	M-3620	http://www.Aurumpharmatech.com/Product/ProductDetails/M_3620
Avantor Inc	BT218670-1G	https://us.vwr.com/store/product/16902257/3-4-diaminopyridine-98
Avantor Inc	BT218670-25G	https://us.vwr.com/store/product/16902257/3-4-diaminopyridine-98
Avantor Inc	BT218670-5G	https://us.vwr.com/store/product/16902257/3-4-diaminopyridine-98
BLD Pharm	BD9613	http://www.bldpharm.com/products/54-96-6.html
Beijing Acemol Technology	AMPD00217	http://www.acemol.com/products/PAMPD00217.shtml
BioChemPartner	BCP22343	http://www.biochempartner.com/54-96-6-BCP22343
BioCrick	BCC5185	http://www.biocrick.com/Amifampridine-BCC5185.html
BioSynth	W-105592	https://www.biosynth.com/en/products/chemical-intermediates/basic-intermediates/products/W-105592.html
BioSynth	J-610017	https://www.biosynth.com/en/products/life-science/other-biochemicals/products/J-610017.html
Boerchem	BC222447	http://www.boerchem.com/?product_41833.html
BroadPharm	BP-12781	http://www.broadpharm.com/web/product.php?catalog=BP-12781
CSNpharm	CSN19508	https://www.csnpharm.com/products/amifampridine.html
Capot Chemical	1295	http://www.capotchem.com/en/1295.htm
Capot Chemical	PubChem1295	http://www.capotchem.com/en/1295.htm
Chem Scene	CS-2778	http://www.chemscene.com/54-96-6.html
ChemMol	1201412	http://www.chemmol.com/chemmol/1201412.html
ChemMol	44006862	http://www.chemmol.com/chemmol/44006862.html
ChemMol	49425879	http://www.chemmol.com/chemmol/49425879.html
ChemMol	81407478	http://www.chemmol.com/chemmol/81407478.html
ChemMol	81412195	http://www.chemmol.com/chemmol/81412195.html
ChemReagents	813047	http://www.chemreagents.com/Product/Product.asp?ProductID=813047
Chemenu	CM108190	http://www.chemenu.com/products/CM108190
Chemspace	CSSB00000752140	https://chem-space.com/CSSB00000752140
Coresyn	CM14022	http://www.coresyn.com/Cas54-96-6.html
Debye Scientific	DB-011105	http://www.debyesci.com/cas_54-96-6.html
Finetech	FT-0614203	http://www.finetechnology-ind.com/prod/detail/pub/54-96-6.html
Glentham Life Sciences	GK1922	http://www.glentham.com/products/product/GK1922/
Hairui	HR1295	http://www.hairuichem.com/en/product/54-96-6.html
Hefei Hirisun Pharmatech Co., Ltd	HR013379	http://www.hirisunpharm.com/54-96-6.html
LGC Standards	MM3483.00	https://www.lgcstandards.com/US/en/p/MM3483.00
Lab Network	LN00007606	https://labnetwork.com/frontend-app/p/#!/moleculedetails/LN00007606
Lumtec	K0349	http://www.lumtec.com.tw/portal_c1_cnt_page.php?owner_num=c1_290785&button_num=c1&folder_id=32214&cnt_id=259189&search_field=&search_word=&search_field2=&search_word2=&search_field3=&search_word3=&bool1=&bool2=&search_type=1&up_page=1
MIC Scientific	HC150250	http://www.michemical.com/mkdetail.php?id=867
Matrix Scientific	11183	https://www.matrixscientific.com/011183.html
MedChem Express	HY-14946	https://www.medchemexpress.com/Amifampridine.html
Molcore	CS22778	https://www.molcore.com/product/54-96-6
MuseChem	I004036	https://www.musechem.com/product/I004036.html
OChem	2159	http://www.ocheminc.com/index.php?act=psearch&pid=006D401
Oakwood	43674	http://www.oakwoodchemical.com/ProductsList.aspx?CategoryID=-2&txtSearch=37550&ExtHyperLink=1
Oakwood	47550	http://www.oakwoodchemical.com/p-57797-047550.aspx
Parchem	28175	http://www.parchem.com/chemical-supplier-distributor/Amifampridine-018175.aspx
R&D Chemicals	D219	http://www.rdchemicals.com/chemicals.php?mode=details&mol_id=6534
Renno Tech	RP00483	http://www.rennotech.com/product_show.asp?id=6873
Sigma-Aldrich	D7148_ALDRICH	https://www.sigmaaldrich.com/catalog/product/aldrich/d7148?utm_source=pubchem&utm_campaign=pubchem_2017&utm_medium=referral
Sinfoo Biotech	S017614	http://www.sinfoobiotech.com/product/1021012
Smolecule	S518476	http://www.smolecule.com/products/s518476
Smolecule	S748722	https://www.smolecule.com/products/s748722
TCI (Tokyo Chemical Industry)	D1149	http://www.tcichemicals.com/eshop/en/us/commodity/D1149/index.html
THE BioTek	bt-259134	https://www.thebiotek.com/product/inhibitors/bt-259134
THE BioTek	bt-308561	https://www.thebiotek.com/product/others/bt-308561
VladaChem	VL141657	https://www.vladachem.com/product.php?products=54-96-6
VladaChem	VL263634-25G	https://www.vladachem.com/product.php?products=54-96-6
Yuhao Chemical	LQ0029	http://www.chemyuhao.com/54-96-6.html
abcr GmbH	AB115247	http://www.abcr.com/shop/en/AB115247
eNovation Chemicals	K02926	https://www.enovationchem.com/ProductDetails.asp?ProductID=K02926
eNovation Chemicals	K15425	https://www.enovationchem.com/ProductDetails.asp?ProductID=K15425
iChemical	EBD541096	http://www.ichemical.com/products/54-96-6.html?utm_source=PubChem&utm_medium=website&utm_campaign=product%20catalogs
mcule	MCULE-5478459845	https://mcule.com/MCULE-5478459845/

PubMed

Title	Date	PubMed
Expression and function of native potassium channel [K(V)alpha1] subunits in terminal arterioles of rabbit.	2001 Aug 1	11483700
Modulation of diaphragm action potentials by K(+) channel blockers.	2001 Feb	11173076
Electrophysiological analysis of the neurotoxic action of a funnel-web spider toxin, delta-atracotoxin-HV1a, on insect voltage-gated Na+ channels.	2001 Feb	11171353
Neurogenic bladder in Lambert-Eaton myasthenic syndrome and its response to 3,4-diaminopyridine.	2001 Jan 15	11166786
Theoretical analysis of the molecular determinants responsible for the K(+) channel blocking by aminopyridines.	2001 Jun 15	11403883
Potassium channel KV alpha1 subunit expression and function in human detrusor muscle.	2002 Apr	11912453
Action of Micrurus dumerilii carinicauda coral snake venom on the mammalian neuromuscular junction.	2002 Feb	11689238
Analysis of the pressor response to the K+ channel inhibitor 4-aminopyridine.	2002 Oct 11	12359274
Treatment for Lambert-Eaton myasthenic syndrome.	2003	12804456
The vascular response to the K+ channel inhibitor 4-aminopyridine in hypertensive rats.	2003 Apr 18	12694813
3,4-DAP-evoked transmitter release in hippocampal slices of aged rats with impaired memory.	2003 Dec 15	14638386
Exacerbation of Lambert-Eaton myasthenic syndrome caused by an L-type Ca2+ channel antagonist.	2003 Jan	12622445
Effect of thymol on kinetic properties of Ca and K currents in rat skeletal muscle.	2003 Jul 15	12864924
Treatment of downbeat nystagmus with 3,4-diaminopyridine: a placebo-controlled study.	2003 Jul 22	12874393
Therapy in myasthenia gravis and Lambert-Eaton myasthenic syndrome.	2003 Jun	12894384
Rational modelling of the voltage-dependent K+ channel inactivation by aminopyridines.	2003 Jun 1	12878310
[Formulation and stability of hospital preparation of 3,4-diaminopyridine capsules].	2003 Nov	14639194
Sternohyoid muscle fatigue properties of dy/dy dystrophic mice, an animal model of merosin-deficient congenital muscular dystrophy.	2003 Oct	12840158
Lambert-eaton myasthenic syndrome: diagnosis and treatment.	2003 Sep	14592920
Effect of 3,4-diaminopyridine on the gravity dependence of ocular drift in downbeat nystagmus.	2004 Aug 24	15326263
Electrophysiological and pharmacological characterization of K+-currents in muscle fibres isolated from the ventral sucker of Fasciola hepatica.	2004 Dec	15648701
Lambert-Eaton myasthenic syndrome.	2004 Feb	15034474
Combination of variable frequency train stimulation and K+ channel blockade to augment skeletal muscle force.	2004 Jun	15218942
Determination of A 3,4-diaminopyridine in plasma by liquid chromatography with electrochemical detection using solid-phase extraction.	2004 Jun 15	15135099
Enhanced brain motor activity in patients with MS after a single dose of 3,4-diaminopyridine.	2004 Jun 8	15184612
Dual effects of nitric oxide in the mouse forced swimming test: possible contribution of nitric oxide-mediated serotonin release and potassium channel modulation.	2004 Mar	15006455
Effect of 3,4-diaminopyridine on the postural control in patients with downbeat nystagmus.	2005 Apr	15826992
NCAM 180 acting via a conserved C-terminal domain and MLCK is essential for effective transmission with repetitive stimulation.	2005 Jun 16	15953420
Cognitive deficits in aged rats correlate with levels of L-arginine, not with nNOS expression or 3,4-DAP-evoked transmitter release in the frontoparietal cortex.	2005 Mar	15695061
Guidelines for the treatment of autoimmune neuromuscular transmission disorders.	2006 Jul	16834699
Potassium channel blockers in multiple sclerosis: neuronal Kv channels and effects of symptomatic treatment.	2006 Jul	16472864
[Fatigue and episodic exhaustion as a feature of multiple sclerosis].	2006 Mar	16585884
Treatment of the gravity dependence of downbeat nystagmus with 3,4-diaminopyridine.	2006 Sep 12	16966567
Altered diaphragm muscle action potentials in Zucker diabetic fatty (ZDF) rats.	2006 Sep 28	16311078
Beneficial effects of 3,4-diaminopyridine on positioning downbeat nystagmus in a circumscribed uvulo-nodular lesion.	2007 Aug	17410325
[Successful treatment of a Lambert-Eaton myasthenic syndrome patient with 3,4-diaminopyridine].	2007 Aug 10	17802724
Stability studies of ionised and non-ionised 3,4-diaminopyridine: hypothesis of degradation pathways and chemical structure of degradation products.	2007 Jan 4	16844337
[Treatment of Lambert-Eaton syndrome with 3,4- diaminopyridine and pyridostigmine].	2007 Jul-Aug	18052623
Potassium initiates vasodilatation induced by a single skeletal muscle contraction in hamster cremaster muscle.	2007 Jun 1	17363384
Autoantibodies in neuromuscular transmission disorders.	2008 Jul	19893659
Mechanisms of neuromodulation as dissected using Sr2+ at motor nerve endings.	2008 Jun	18385484
Therapeutic strategies in congenital myasthenic syndromes.	2008 Oct	19019305
Lambert-Eaton myasthenic syndrome: search for alternative autoimmune targets and possible compensatory mechanisms based on presynaptic calcium homeostasis.	2008 Sep 15	18653248
[A case of Lambert-Eaton myasthenic syndrome with small cell lung cancer, treated with 3,4-diaminopyridine].	2009 Jan	19198242
Skeletal muscle contraction-induced vasodilator complement production is dependent on stimulus and contraction frequency.	2009 Jul	19465553
Efficacy of 3,4-diaminopyridine and pyridostigmine in the treatment of Lambert-Eaton myasthenic syndrome: a randomized, double-blind, placebo-controlled, crossover study.	2009 Jul	19357643
Management of myasthenic conditions: nonimmune issues.	2009 Oct	19593127
3,4-diaminopyridine safety in clinical practice: an observational, retrospective cohort study.	2010 Jun	20058019

Patents

Sample Use Guides

In Vivo Use Guide

Amifampridine (Firdapse) should be given in divided doses, three or four times a day. The recommended starting dose is 15 mg a day, which can be increased in 5 mg increments every 4 to 5 days, to a maximum of 60 mg per day. No single dose should exceed 20 mg. Tablets are to be taken with food. For oral use only.

Route of Administration: Oral

In Vitro Use Guide

In nerve terminal regions of axons in rat olfactory cortex in the presence of tetraethylammonium (TEA, 5 mM) and Cd2+ (100 uM), the K(+)-component was depressed by Amifampridine (3,4-DAP; 1 to 20 uM; IC50 2.0 +/- 0.4 uM).

Substance Class	Chemical Created by `admin` on `Fri Dec 15 16:22:23 GMT 2023` Edited by `admin` on `Fri Dec 15 16:22:23 GMT 2023`
Record UNII	RU4S6E2G0J
Record Status	`Validated (UNII)`
Record Version

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Name

Type

Language

AMIFAMPRIDINE

Official Name

English

AMIFAMPRIDINE [MI]

Common Name

English

3,4 DIAMINOPYRIDINE

Systematic Name

English

AMIFAMPRIDINE [ORANGE BOOK]

Common Name

English

NSC-521760

Code

English

amifampridine [INN]

Common Name

English

PYRIDINE, 3,4-DIAMINO-

Systematic Name

English

DAP

Common Name

English

AMIFAMPRIDINE [MART.]

Common Name

English

3,4-DIAMINOPYRIDINE

Systematic Name

English

Amifampridine [WHO-DD]

Common Name

English

AMIFAMPRIDINE [USAN]

Common Name

English

3,4 DIAMINOPYRIDINE [VANDF]

Common Name

English

3,4-DAP

Code

English

DYNAMINE

Brand Name

English

RUZURGI

Brand Name

English

AMIFAMPRIDINE [EMA EPAR]

Common Name

English

Classification Tree Code System Code

FDA ORPHAN DRUG

56090