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| Primary information |
|---|
| ID | 15939 |
| Therapeutic ID | Th1653 |
| Protein Name | Agalsidase alfa |
| Sequence | >Th1653_Agalsidase_alfa
LDNGLARTPTMGWLHWERFMCNLDCQEEPDSCISEKLFMEMAELMVSEGWKDAGYEYLCIDDCWMAPQRDSEGRLQADPQRFPHGIRQLANYVHSKGLKLGIYADVGNKTCAGFPGSFGYYDIDAQTFADWGVDLLKFDGCYCDSLENLADGYKHMSLALNRTGRSIVYSCEWPLYMWPFQKPNYTEIRQYCNHWRNFADIDDSWKSIKSILDWTSFNQERIVDVAGPGGWNDPDMLVIGNFGLSWNQQVTQMALWAIMAAPLFMSNDLRHISPQAKALLQDKDVIAINQDPLGKQGYQLRQGDNFEVWERPLSGLAWAVAMINRQEIGGPRSYTIAVASLGKGVACNPACFITQLLPVKRKLGFYEWTSRLRSHINPTGTVLLQLENTMQMSLKDLL
|
| Molecular Weight | 45351.6 |
| Chemical Formula | C2029H3080N544O587S27 |
| Isoelectric Point | NA |
| Hydrophobicity | NA |
| Melting point | NA |
| Half-life | The elimination half life was 108 ± 17 minutes for males and 89 ± 28 minutes for females.[L16398] |
| Description | Agalsidase alfa is a recombinant human a-galactosidase A similar to [agalsidase beta]. While patients generally do not experience a clinically significant difference in outcomes between the two drugs, some patients may experience greater benefit with agalsidase beta.[A220228,A220233] Use of agalsidase beta has decreased in Europe, in favor of agalsidase alfa, after a contamination event in 2009.[A220343] Agalsidase alfa was granted EMA approval on 3 August 2001.[L16413] |
| Indication/Disease | Agalsidase alfa is indicated in the treatment of Fabry disease.[L16398] |
| Pharmacodynamics | Agalsidase alfa is a recombinant human a-galactosidase A used as enzyme replacement therapy in the treatment of Fabry disease.[L16398] It has a long duration of action and a wide therapeutic index.[L16398] Patients should be counselled regarding the risk of infusion related reactions and hypersensitivity.[L16398] |
| Mechanism of Action | a-galactosidase A is uptaken by cells via the mannose 6 phosphate receptor.[A220323] Agalsidase alfa hydrolyzes globotriaosylceramide and other glycosphingolipids that would normally be hydrolyzed by endogenous a-galactosidase A.[L16398] Preventing the accumulation of glycosphingolipids prevents or reduces the severity of manifestations of Fabry disease such as renal failure, cardiomyopathy, or cerebrovascular events.[L16398] |
| Toxicity | Data regarding overdoses of agalsidase alfa are not readily available.[L16398] Patients experiencing an overdose of agalsidase alfa may experience an increased incidence and severity of adverse effects. Overdose can be managed through the use of symptomatic and supportive measures. |
| Metabolism | Data regarding the metabolism of agalsidase alfa is not readily available.[L16398] However, protein drugs are expected to be degraded by proteases and other catalytic enzymes to smaller peptides and amino acids.[A182009] |
| Absorption | A dose of agalsidase alfa in non end stage renal disease patients reaches a Cmax of 3710 ± 855 U/mL with an AUC of 256,958 ± 63,499 min |
| The volume of distribution at steady state in non end stage renal disease patients was approximately 17% of body weight regardless of sex.[A220338,L16398] |
| Clearance | The clearance for doses of 0.007-0.2 mg/kg were 2.66 mL/min/kg for males and 2.10 mL/min/kg for females.[L16398] |
| Categories | Enzymes |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | Globotriaosylceramide |
| Brand Name | NA |
| Company | NA |
| Brand Description | NA |
| Prescribed For | NA |
| Chemical Name | NA |
| Formulation | NA |
| Physical Appearance | NA |
| Route of Administration | NA |
| Recommended Dosage | NA |
| Contraindication | NA |
| Side Effects | NA |
| Useful Link 1 | Link |
| Useful Link 2 | NA |
| Remarks | NA |