Amifampridine Phosphate - CAS 446254-47-3
Catalog number: 446254-47-3
Category: Inhibitor
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Molecular Formula:
Molecular Weight:
Amifampridine is a drug, predominantly used to treat many of the congenital myasthenic syndromes, particularly those with defects in choline acetyltransferase, downstream kinase 7, and those where any kind of defect causes "fast channel" behaviour of the acetylcholine receptor.
White Solid
phosphoric acid;pyridine-3,4-diamine;
Soluble in DMSO
Store at -20 °C
Predominantly in the treatment of a number of rare muscle diseases
Quality Standard:
Enterprise standard
Shelf Life:
As supplied, 2 years from the QC date provided on the Certificate of Analysis, when stored properly.
Canonical SMILES:
1.Improvement in neuromuscular transmission in myasthenia gravis by 3,4-diaminopyridine.
Lundh H;Nilsson O;Rosén I Eur Arch Psychiatry Neurol Sci. 1985;234(6):374-7.
3,4-Diaminopyridine (3,4-DAP), a potent potentiator of action potential evoked release of acetylcholine from presynaptic terminals in the neuromuscular junction was given i.v. and p.o. to two patients with myasthenia gravis. Effects were monitored electrophysiologically by repetitive nerve stimulation and by standardized clinical testing. Administration of 8 mg and 9 mg 3,4-DAP i.v. produced a clear improvement in the neuromuscular transmission after approximately 20 min. When 3,4-DAP was given p.o. 24 mg was shown to be effective. At a dosage of 18-24 mg p.o. 3,4-DAP significantly potentiated the effect of the cholinesterase inhibitor pyridostigmine at an optimal dose. The maximal effect of 3,4-DAP p.o. was obtained after 2.5-3 h. No significant CNS side-effects were found which is in contrast to those reported for 4-aminopyridine. The results suggest that 3,4-DAP may be useful as an addition to the conventional treatment with cholinesterase inhibitors when immunosuppressive treatment is considered contraindicated or when it has not yet reached its full effect.
2.Amifampridine phosphate (Firdapse(®)) is effective and safe in a phase 3 clinical trial in LEMS.
Oh SJ;Shcherbakova N;Kostera-Pruszczyk A;Alsharabati M;Dimachkie M;Blanco JM;Brannagan T;Lavrnić D;Shieh PB;Vial C;Meisel A;Komoly S;Schoser B;Sivakumar K;So Y;LEMS Study Group Muscle Nerve. 2016 May;53(5):717-25. doi: 10.1002/mus.25070. Epub 2016 Mar 3.
OBJECTIVE: ;We evaluated the efficacy and safety of amifampridine phosphate (Firdapse(®)) for symptomatic treatment in Lambert-Eaton myasthenic syndrome (LEMS).;METHODS: ;Phase 3, randomized, double-blind, study. Patients were treated initially with amifampridine phosphate for 7-91 days, followed by randomization to continue amifampridine phosphate for 14 days or placebo (7-day taper, 7-day placebo). The primary efficacy endpoints were changes from baseline at day 14 in Quantitative Myasthenia Gravis and Subject Global Impression scores.;RESULTS: ;The coprimary efficacy end points and 1 of the secondary efficacy end points were met, showing a significant benefit of aminfampridine phosphate over placebo at Day 14. All 5 primary, secondary, and tertiary endpoints achieved statistical significance at Day 8. Amifampridine phosphate was well tolerated; the most common adverse events were oral and digital paresthesias, nausea, and headache.;CONCLUSIONS: ;This study provides Class I evidence of efficacy of amifampridine phosphate as a symptomatic treatment for LEMS.;© 2016 Wiley Periodicals, Inc.
3.Five years experience on 3,4-diaminopyridine phosphate in Lambert-Eaton syndrome: Case reports.
Portaro S;Brizzi T;Sinicropi S;Cacciola A;De Cola MC;Bramanti A;Milardi D;Lupica A;Bramanti P;Toscano A;Rodolico C Medicine (Baltimore). 2017 Sep;96(38):e7839. doi: 10.1097/MD.0000000000007839.
RATIONALE: ;To report our experience on 7 patients (4 males and 3 females), affected by nonparaneoplastic Lambert-Eaton myasthenic syndrome, treated with 3,4-diaminopyridine phosphate (3,4-DAPP) either alone or in combination with other immunosuppressants or steroids.;PATIENT CONCERNS: ;Patients have been evaluated at specific timepoints (ie, baseline and last 5 year follow-up), with neurological examination, autoantibodies against presynaptic voltage-gated Cav2.1 (P/Q type) calcium ion channel (VGCC) dosage, neurophysiological evaluation focusing on the increased amplitude of the compound muscle action potential (cMAP) after maximum voluntary effort, quantitative myasthenia gravis (QMG) and activities of daily living scales, and autonomic nervous system involvement evaluation.;OUTCOMES: ;Five out of 7 patients presented a clinical improvement persisting at last 5-year follow-up; 2 out of them improved taking only 3,4-DAPP at the maximal dosage, whereas the remaining received concomitant medications, such as prednisone and azathioprine. However, the clinical amelioration was not statistically significant. No one of the patients reported severe adverse events, except one, complaining of transient chin and perioral paresthesias.
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