AMIFAMPRIDINE PHOSPHATE

Details

Stereochemistry	ACHIRAL
Molecular Formula	C5H7N3.H3O4P
Molecular Weight	207.1244
Optical Activity	NONE
Defined Stereocenters	0 / 0
E/Z Centers	0
Charge	0

SHOW SMILES / InChI

Structure Search

SMILES

OP(O)(O)=O.NC1=CC=NC=C1N

InChI

InChIKey=KAICRBBQCRKMPO-UHFFFAOYSA-N

InChI=1S/C5H7N3.H3O4P/c6-4-1-2-8-3-5(4)7;1-5(2,3)4/h1-3H,7H2,(H2,6,8);(H3,1,2,3,4)

HIDE SMILES / InChI

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 555		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 8583	Firdapse Approved Use Symptomatic treatment of Lambert-Eaton myasthenic syndrome (LEMS) in adults. Launch Date 2009

C_max

Value	Dose	Analyte	Population
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
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
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
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
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
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
2.28 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
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
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 Health Status: healthy Age Group: adult 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 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 Health Status: unhealthy Age Group: adult Sex: M+F 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 2252 inducer 1087	inhibitor 2438	inhibitor 2507	inhibitor 2217

OverviewOther

Other Inhibitor	Other Substrate	Other Inducer
Kv1.3 15 Kv1.5 27 P-gp 1741 OATP1B3 961 CYP1A2 2153 CYP2A6 879 CYP2B6 926 CYP2C8 1057 CYP2C19 2057 CYP2E1 1060	P-gp 2052 CYP450 59 FMO 34	CYP1A2 832 CYP2B6 669

Drug as perpetrator

Target	Modality	Activity	Metabolite
OATP1B3 970	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no [Inhibition 30 uM]
CYP1A2 2333	inducer 1966 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no	3-N-acetyl-BMN125 2
CYP1A2 2333	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no
CYP2A6 911	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no
CYP2B6 1160	inducer 1966 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no	3-N-acetyl-BMN125 2
CYP2B6 1160	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no
CYP2C19 2141	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no
CYP2C8 1117	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no
CYP2C9 2497	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no
CYP2D6 2546	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no
CYP2E1 1146	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no
CYP3A4 2633	inducer 1966 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no	3-N-acetyl-BMN125 2
CYP3A4 2633	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no
Kv1.3 30	inhibitor 4027 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 42	inhibitor 4027 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 1932	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no
Kv1.5 42	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=25 Page: 25.0	yes [IC50 490.9 uM]
Kv1.3 30	inhibitor 4027 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=25 Page: 25.0	yes [IC50 524.8 uM]
CYP1A2 2333	inducer 1966 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	yes
CYP2B6 1160	inducer 1966 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	yes
CYP3A4 2633	inducer 1966 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 59	substrate 4014 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no
FMO 34	substrate 4014 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=27 Page: 27.0	no
P-gp 2052	substrate 4014 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 2263	inhibitor 2237 Sources: https://www.accessdata.fda.gov/drugsatfda_docs/nda/2018/208078Orig1s000PharmR.pdf#page=26 Page: 26.0
hERG 2263	inhibitor 2237 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
Aaron Chemicals	AR003J4H	http://www.aaronchem.com/54-96-6
abcr GmbH	AB115247	http://www.abcr.com/shop/en/AB115247
AbMole Bioscience	M5406	http://www.abmole.com/products/amifampridine.html
Acadechem	ACDS-033271	http://www.acadechemical.com/product/107970
Acadechem	ACDX-013489	http://acadechemical.com/product/88181
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
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
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
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	J-610017	https://www.biosynth.com/en/products/life-science/other-biochemicals/products/J-610017.html
BioSynth	W-105592	https://www.biosynth.com/en/products/chemical-intermediates/basic-intermediates/products/W-105592.html
BLD Pharm	BD9613	http://www.bldpharm.com/products/54-96-6.html
Boerchem	BC222447	http://www.boerchem.com/?product_41833.html
BroadPharm	BP-12781	http://www.broadpharm.com/web/product.php?catalog=BP-12781
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
Chemenu	CM108190	http://www.chemenu.com/products/CM108190
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
Chemspace	CSSB00000752140	https://chem-space.com/CSSB00000752140
Coresyn	CM14022	http://www.coresyn.com/Cas54-96-6.html
CSNpharm	CSN19508	https://www.csnpharm.com/products/amifampridine.html
Debye Scientific	DB-011105	http://www.debyesci.com/cas_54-96-6.html
eNovation Chemicals	K02926	https://www.enovationchem.com/ProductDetails.asp?ProductID=K02926
eNovation Chemicals	K15425	https://www.enovationchem.com/ProductDetails.asp?ProductID=K15425
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
iChemical	EBD541096	http://www.ichemical.com/products/54-96-6.html?utm_source=PubChem&utm_medium=website&utm_campaign=product%20catalogs
Lab Network	LN00007606	https://labnetwork.com/frontend-app/p/#!/moleculedetails/LN00007606
LGC Standards	MM3483.00	https://www.lgcstandards.com/US/en/p/MM3483.00
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
Matrix Scientific	11183	https://www.matrixscientific.com/011183.html
mcule	MCULE-5478459845	https://mcule.com/MCULE-5478459845/
MedChem Express	HY-14946	https://www.medchemexpress.com/Amifampridine.html
MIC Scientific	HC150250	http://www.michemical.com/mkdetail.php?id=867
Molcore	CS22778	https://www.molcore.com/product/54-96-6
MuseChem	I004036	https://www.musechem.com/product/I004036.html
Oakwood	43674	http://www.oakwoodchemical.com/ProductsList.aspx?CategoryID=-2&txtSearch=37550&ExtHyperLink=1
Oakwood	47550	http://www.oakwoodchemical.com/p-57797-047550.aspx
OChem	2159	http://www.ocheminc.com/index.php?act=psearch&pid=006D401
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

PubMed

Title	Date	PubMed
3,4-diaminopyridine safety in clinical practice: an observational, retrospective cohort study.	2010-06	20058019
Quantification of 4-aminopyridine in plasma by capillary electrophoresis with electrokinetic injection.	2010-02-01	19962357
Gold and silver nanoparticles conjugated with heparin derivative possess anti-angiogenesis properties.	2009-11-11	19822927
3,4-Diaminopyridine is more effective than placebo in a randomized, double-blind, cross-over drug study in LEMS.	2009-11	19722254
Management of myasthenic conditions: nonimmune issues.	2009-10	19593127
Skeletal muscle contraction-induced vasodilator complement production is dependent on stimulus and contraction frequency.	2009-07	19465553
Efficacy of 3,4-diaminopyridine and pyridostigmine in the treatment of Lambert-Eaton myasthenic syndrome: a randomized, double-blind, placebo-controlled, crossover study.	2009-07	19357643
Mutations in LAMB2 causing a severe form of synaptic congenital myasthenic syndrome.	2009-03	19251977
[A case of Lambert-Eaton myasthenic syndrome with small cell lung cancer, treated with 3,4-diaminopyridine].	2009-01	19198242
[Treatment approach to congenital myasthenic syndrome in a patient with acetylcholine receptor deficiency].	2009-01	19172815
Long-lasting in vivo inotropic effects of the K(+) channel blocker 3,4-diaminopyridine during fatigue-inducing stimulation.	2008-12	19016549
[Pharmacotherapy of central oculomotor disorders].	2008-12	18633586
Therapeutic strategies in congenital myasthenic syndromes.	2008-10	19019305
Lambert-Eaton myasthenic syndrome: search for alternative autoimmune targets and possible compensatory mechanisms based on presynaptic calcium homeostasis.	2008-09-15	18653248
Brain structural and functional abnormalities in mood disorders: implications for neurocircuitry models of depression.	2008-09	18704495
Autoantibodies in neuromuscular transmission disorders.	2008-07	19893659
Onset dynamics of type A botulinum neurotoxin-induced paralysis.	2008-06	18551355
Mechanisms of neuromodulation as dissected using Sr2+ at motor nerve endings.	2008-06	18385484
L-type Ca2+ channel function is linked to dystrophin expression in mammalian muscle.	2008-03-12	18516256
Inotropic effects of the K+ channel blocker 3,4-diaminopyridine on fatigued diaphragm muscle.	2008-01-01	17881299
[Lambert-Eaton Myasthenic Syndrome associated with vocal cord carcinoma].	2008-01	18342060
[Treatment of Lambert-Eaton syndrome with 3,4- diaminopyridine and pyridostigmine].	2007-12-07	18052623
[Successful treatment of a Lambert-Eaton myasthenic syndrome patient with 3,4-diaminopyridine].	2007-08-10	17802724
Childhood myasthenia: clinical subtypes and practical management.	2007-08	17635211
Beneficial effects of 3,4-diaminopyridine on positioning downbeat nystagmus in a circumscribed uvulo-nodular lesion.	2007-08	17410325
Myasthenia gravis and Lambert-Eaton myasthenic syndrome in the same patient.	2007-07	17206662
Potassium initiates vasodilatation induced by a single skeletal muscle contraction in hamster cremaster muscle.	2007-06-01	17363384
Neuromuscular paralysis and recovery in mice injected with botulinum neurotoxins A and C.	2007-05	17561839
Molecular docking study of the binding of aminopyridines within the K+ channel.	2007-05	17340113
The therapy of congenital myasthenic syndromes.	2007-04	17395135
Stability studies of ionised and non-ionised 3,4-diaminopyridine: hypothesis of degradation pathways and chemical structure of degradation products.	2007-01-04	16844337
Inotropic effects of the K+ channel blocker 3,4-diaminopyridine: differential responses of rat soleus and extensor digitorum longus.	2006-12	17190034
Altered diaphragm muscle action potentials in Zucker diabetic fatty (ZDF) rats.	2006-09-28	16311078
Treatment of the gravity dependence of downbeat nystagmus with 3,4-diaminopyridine.	2006-09-12	16966567
Extrusion of Ca2+ from mouse motor terminal mitochondria via a Na+-Ca2+ exchanger increases post-tetanic evoked release.	2006-08-01	16613870
Pre- and post-synaptic abnormalities associated with impaired neuromuscular transmission in a group of patients with 'limb-girdle myasthenia'.	2006-08	16870884
Guidelines for the treatment of autoimmune neuromuscular transmission disorders.	2006-07	16834699
Available treatment options for the management of Lambert-Eaton myasthenic syndrome.	2006-07	16805718
Potassium channel blockers in multiple sclerosis: neuronal Kv channels and effects of symptomatic treatment.	2006-07	16472864
No benefit of 3,4-diaminopyridine in essential tremor: a placebo-controlled crossover study.	2006-06-13	16769957
The effects of presynaptic calcium channel modulation by roscovitine on transmitter release at the adult frog neuromuscular junction.	2006-06	16820010
Fetal exposure to 3,4-diaminopyridine in a pregnant woman with congenital myasthenia syndrome.	2006-04	16537815
[Pathophysiology and treatment of fatigue in multiple sclerosis].	2006-03	16585886
[Fatigue and episodic exhaustion as a feature of multiple sclerosis].	2006-03	16585884
NCAM 180 acting via a conserved C-terminal domain and MLCK is essential for effective transmission with repetitive stimulation.	2005-06-16	15953420
Taicatoxin inhibits the calcium-dependent slow motility of mammalian outer hair cells.	2005-05	15855042
Treatment for Lambert-Eaton myasthenic syndrome.	2005-04-18	15846654
Effect of 3,4-diaminopyridine on the postural control in patients with downbeat nystagmus.	2005-04	15826992
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.	2005-03	15695061
[Lambert-Eaton myasthenic syndrome: diagnosis and treatment].	2001-11	15775666

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).

Name

Type

Language

FIRDAPSE

Preferred Name

English

AMIFAMPRIDINE PHOSPHATE

Official Name

English

Amifampridine phosphate [WHO-DD]

Common Name

English

3,4-DIAMINOPYRIDINE PHOSPHATE

Systematic Name

English

AMIFAMPRIDINE PHOSPHATE [MI]

Common Name

English

ZENAS

Brand Name

English

3,4-DAPP

Code

English

AMIFAMPRIDINE PHOSPHATE [MART.]

Common Name

English

AMIFAMPRIDINE PHOSPHATE [EMA EPAR]

Common Name

English

AMIFAMPRIDINE PHOSPHATE [USAN]

Common Name

English

AMIFAMPRIDINE PHOSPHATE [ORANGE BOOK]

Common Name

English

DAPP

Code

English

3,4-PYRIDINEDIAMINE, PHOSPHATE (1:1)

Systematic Name

English

Classification Tree Code System Code

EMA ASSESSMENT REPORTS

FIRDAPSE (AUTHORIZED: LAMBERT-EATON MYASTHENIC SYNDROME)