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| Primary information |
|---|
| ID | 10332 |
| Therapeutic ID | Th1047 |
| Protein Name | Alpha-1-proteinase inhibitor |
| Sequence | >Th1047_Alpha-1-proteinase_inhibitor
EDPQGDAAQKTDTSHHDQDHPTFNKITPNLAEFAFSLYRQLAHQSNSTNIFFSPVSIATAFAMLSLGTKADTHDEILEGLNFNLTEIPEAQIHEGFQELLRTLNQPDSQLQLTTGNGLFLSEGLKLVDKFLEDVKKLYHSEAFTVNFGDTEEAKKQINDYVEKGTQGKIVDLVKELDRDTVFALVNYIFFKGKWERPFEVKDTEEEDFHVDQVTTVKVPMMKRLGMFNIQHCKKLSSWVLLMKYLGNATAIFFLPDEGKLQHLENELTHDIITKFLENEDRRSASLHLPKLSITGTYDLKSVLGQLGITKVFSNGADLSGVTEEAPLKLSKAVHKAVLTIDEKGTEAAGAMFLEAIPMSIPPEVKFNKPFVFLMIEQNTKSPLFMGKVVNPTQK
|
| Molecular Weight | 44324.5 |
| Chemical Formula | C2001H3130N514O601S10 |
| Isoelectric Point | 5.37 |
| Hydrophobicity | -0.302 |
| Melting point | 59 |
| Half-life | NA |
| Description | Human alpha-1 proteinase inhibitor or alpha-1-antitrypsin, prepared from human plasma via Cohn alcohol fractionation followed by PEG and zinc chloride fractionation. |
| Indication/Disease | For treatment of panacinar emphysema. |
| Pharmacodynamics | Prevents excessive accumulation of active neutrophil elastase and consequent proteolysis of elastin tissues in alveolar lung structures. This prevents the development of emphysema. |
| Mechanism of Action | Alpha-1 proteinase inhibitor is a serine protease inhibitor (Serpin). Its primary mechanism is inhibiting the action of the serine protease called elastase (also plasmin and thrombin) in the lungs. The reactive center loop (RCL) of alpha-1 proteinase inhibitor extends out from the body of the protein and directs binding to the target protease. The protease cleaves the serpin at the reactive site, establishing a covalent linkage between the carboxyl group of the serpin reactive site and the serine hydroxyl of the protease. The resulting inactive serpin-protease complex is highly stable. |
| Toxicity | NA |
| Metabolism | NA |
| Absorption | NA |
| 5618 ± 1618 mL [Aralast] |
| Clearance | 940 ± 275 mL/day [Patients with congenital deficiency with single IV infusion of 60mg/kg] |
| Categories | Acute-Phase Proteins, Alpha-Globulins, Amino Acids, Peptides, and Proteins, Antifibrinolytic Agents, Blood and Blood Forming Organs, Blood Proteins, Enzyme Inhibitors, Enzymes, Enzymes and Coenzymes, Globulins, Glycoproteins, Hemostatics, Human alpha-1 Proteinase Inhibitor, Peptides, Protease Inhibitors, Proteinase Inhibitors, Proteins, Serine Protease Inhibitors, Serpins, Serum Globulins, Trypsin Inhibitors |
| Patents Number | NA |
| Date of Issue | NA |
| Date of Expiry | NA |
| Drug Interaction | NA |
| Target | NA |
| Brand Name | Glassia |
| Company | Takeda, Baxalta US Inc. |
| Brand Description | Takeda, Baxalta US Inc. |
| Prescribed For | treat the symptoms of Alpha-1 Antitrypsin Deficiency. |
| Chemical Name | NA |
| Formulation | GLASSIA is prepared from human plasma obtained from US-licensed plasma collection centers by a modified version of the cold ethanol fractionation process and the Alpha1 -PI is then purified using chromatographic methods. |
| Physical Appearance | sterile, ready to use, liquid preparation of purified human alpha1 -proteinase inhibitor |
| Route of Administration | intravenous |
| Recommended Dosage | Administer 60 mg/kg body weight of GLASSIA once weekly by intravenous infusion. |
| Contraindication | GLASSIA is contraindicated in: immunoglobulin A (IgA) deficient patients with antibodies against IgA. individuals with a history of anaphylaxis or other severe systemic reaction to Alpha11-PI products. |
| Side Effects | hives, difficulty breathing, swelling of your face, lips, tongue, or throat, hives, wheezing, lightheadedness, fever, chills, body aches, flu symptoms, sores in your mouth and throat, pain or burning when you urinate, chest pain or tightness, and vision changes |
| Useful Link 1 | Link |
| Useful Link 2 | NA |
| Remarks | NA |