Alpha-1-proteinase inhibitor
Identification
- Summary
Alpha-1-proteinase inhibitor is a purified form of human alpha-1 proteinase inhibitor used to treat emphysema patients with associated alpha-1 proteinase inhibitor deficiency.
- Brand Names
- Aralast, Glassia, Prolastin, Prolastin-C, Respreeza, Zemaira
- Generic Name
- Alpha-1-proteinase inhibitor
- DrugBank Accession Number
- DB00058
- Background
Human alpha-1 proteinase inhibitor or alpha-1-antitrypsin, prepared from human plasma via Cohn alcohol fractionation followed by PEG and zinc chloride fractionation.
- Type
- Biotech
- Groups
- Approved
- Biologic Classification
- Protein Based Therapies
Other protein based therapies - Protein Structure
- Protein Chemical Formula
- C2001H3130N514O601S10
- Protein Average Weight
- 44324.5 Da
- Sequences
>DB00058 sequence EDPQGDAAQKTDTSHHDQDHPTFNKITPNLAEFAFSLYRQLAHQSNSTNIFFSPVSIATA FAMLSLGTKADTHDEILEGLNFNLTEIPEAQIHEGFQELLRTLNQPDSQLQLTTGNGLFL SEGLKLVDKFLEDVKKLYHSEAFTVNFGDTEEAKKQINDYVEKGTQGKIVDLVKELDRDT VFALVNYIFFKGKWERPFEVKDTEEEDFHVDQVTTVKVPMMKRLGMFNIQHCKKLSSWVL LMKYLGNATAIFFLPDEGKLQHLENELTHDIITKFLENEDRRSASLHLPKLSITGTYDLK SVLGQLGITKVFSNGADLSGVTEEAPLKLSKAVHKAVLTIDEKGTEAAGAMFLEAIPMSI PPEVKFNKPFVFLMIEQNTKSPLFMGKVVNPTQK
Download FASTA Format- Synonyms
- Alfa 1-proteinase inhibitor (human)
- Alfa-1-antitripsina
- Alfa1 antitrypsin
- Alpha 1-antitrypsin
- Alpha 1-Proteinase Inhibitor
- Alpha 1-proteinase inhibitor (human)
- Alpha 1-proteinase inhibitor, human
- Alpha-1 protease inhibitor
- Alpha-1 proteinase inhibitor (human)
- Alpha-1-antiproteinase
- Alpha-1-antitrypsin
- Alpha-1-proteinase Inhibitor (human)
- Alpha-1-proteinase inhibitor human
- Alpha-1-proteinase Inhibitor, Human
- Alpha-1-proteinase inhibitor,human
- Alpha1-proteinase Inhibitor
- Alpha1-proteinase inhibitor (human)
- alpha1-proteinase inhibitor human
- API
- External IDs
- 232-924-7
Pharmacology
- Indication
For chronic augmentation and maintenance therapy in individuals with alpha1-proteinase inhibitor (A1-PI) deficiency and clinical evidence of emphysema.
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- Contraindications & Blackbox Warnings
Avoid life-threatening adverse drug eventsImprove clinical decision support with information on contraindications & blackbox warnings, population restrictions, harmful risks, & more.Avoid life-threatening adverse drug events & improve clinical decision support.- 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.
Target Actions Organism ANeutrophil elastase inhibitorHumans - Absorption
Not Available
- Volume of distribution
- 5632 ± 2006 mL [ARALAST NP]
- 5618 ± 1618 mL [Aralast]
- Protein binding
Not Available
- Metabolism
- Not Available
- Route of elimination
Not Available
- Half-life
Not Available
- Clearance
- 940 +/- 275 mL/day [Patients with congenital α1-PI deficiency with single IV infusion of 60 mg/kg]
- Adverse Effects
Improve decision support & research outcomesWith structured adverse effects data, including: blackbox warnings, adverse reactions, warning & precautions, & incidence rates.Improve decision support & research outcomes with our structured adverse effects data.- Toxicity
Not Available
- Pathways
- Not Available
- Pharmacogenomic Effects/ADRs
- Not Available
Interactions
- Drug Interactions
- This information should not be interpreted without the help of a healthcare provider. If you believe you are experiencing an interaction, contact a healthcare provider immediately. The absence of an interaction does not necessarily mean no interactions exist.
Drug Interaction Integrate drug-drug
interactions in your softwareAlbutrepenonacog alfa Alpha-1-proteinase inhibitor may increase the thrombogenic activities of Albutrepenonacog alfa. Alteplase The therapeutic efficacy of Alpha-1-proteinase inhibitor can be decreased when used in combination with Alteplase. Aminocaproic acid Alpha-1-proteinase inhibitor may increase the thrombogenic activities of Aminocaproic acid. Andexanet alfa Alpha-1-proteinase inhibitor may increase the thrombogenic activities of Andexanet alfa. Anistreplase The therapeutic efficacy of Alpha-1-proteinase inhibitor can be decreased when used in combination with Anistreplase. Anti-inhibitor coagulant complex Alpha-1-proteinase inhibitor may increase the thrombogenic activities of Anti-inhibitor coagulant complex. Antihemophilic factor (recombinant), PEGylated Alpha-1-proteinase inhibitor may increase the thrombogenic activities of Antihemophilic factor (recombinant), PEGylated. Antihemophilic factor human Alpha-1-proteinase inhibitor may increase the thrombogenic activities of Antihemophilic factor human. Antihemophilic factor, human recombinant Alpha-1-proteinase inhibitor may increase the thrombogenic activities of Antihemophilic factor, human recombinant. Aprotinin Alpha-1-proteinase inhibitor may increase the thrombogenic activities of Aprotinin. 
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- No interactions found.
Products
Drug product information from 10+ global regionsOur datasets provide approved product information including:dosage, form, labeller, route of administration, and marketing period.Access drug product information from over 10 global regions.- International/Other Brands
- Aralast (Baxter) / Prolastin (Talecris Biotherapeutics C formerly Bayer)
- Brand Name Prescription Products
Name Dosage Strength Route Labeller Marketing Start Marketing End Region Image Alpha1-proteinase Inhibitor (human) Powder, for solution 500 mg / vial Intravenous Grifols Therapeutics Llc Not applicable Not applicable Canada 
Alpha1-proteinase Inhibitor (human) Powder, for solution 1 g / vial Intravenous Grifols Therapeutics Llc Not applicable Not applicable Canada Aralast NP Kit 16 mg/1mL Intravenous Baxalta u.s. Inc. 2002-12-23 Not applicable US 
Aralast NP Kit 16 mg/1mL Intravenous Baxter Laboratories 2010-09-01 2014-11-09 US Aralast NP Kit 16 mg/1mL Intravenous Baxter Laboratories 2010-09-01 2014-11-09 US Aralast NP Kit 16 mg/1mL Intravenous Baxalta u.s. Inc. 2002-12-23 Not applicable US Aralast NP Kit 16 mg/1mL Intravenous Baxter Laboratories 2010-04-19 2014-11-09 US Glassia Injection, solution 1 g/50mL Intravenous Baxalta Usa Inc. 2010-07-01 Not applicable US Prolastin Inj 25.0mg/ml Powder, for solution 25 mg / mL Intravenous Cutter Biological, Division Of Miles Lab Inc. 1988-12-31 1998-09-25 Canada Prolastin-C 1000 mg/20mL Intravenous GRIFOLS USA, LLC 1987-12-02 Not applicable US
Categories
- ATC Codes
- B02AB02 — Alfa1 antitrypsin
- Drug Categories
- Acute-Phase Proteins
- Alpha-Globulins
- Amino Acids, Peptides, and Proteins
- Antifibrinolytic Agents
- Blood and Blood Forming Organs
- Blood Proteins
- Carbohydrates
- Enzyme Inhibitors
- Enzymes
- Enzymes and Coenzymes
- Globulins
- Glycoconjugates
- Glycoproteins
- Hemostatics
- Human alpha-1 Proteinase Inhibitor
- Peptides
- Protease Inhibitors
- Proteinase Inhibitors
- Proteins
- Serine Protease Inhibitors
- Serpins
- Serum Globulins
- Trypsin Inhibitors
- Chemical TaxonomyProvided by Classyfire
- Description
- Not Available
- Kingdom
- Organic Compounds
- Super Class
- Organic Acids
- Class
- Carboxylic Acids and Derivatives
- Sub Class
- Amino Acids, Peptides, and Analogues
- Direct Parent
- Peptides
- Alternative Parents
- Not Available
- Substituents
- Not Available
- Molecular Framework
- Not Available
- External Descriptors
- Not Available
- Affected organisms
- Humans and other mammals
Chemical Identifiers
- UNII
- F43I396OIS
- CAS number
- 9041-92-3
References
- Synthesis Reference
Michael H. Coan, William J. Brockway, "Method of preparing alpha-1-proteinase inhibitor." U.S. Patent US4379087, issued December, 1968.
US4379087- General References
- Karnaukhova E, Ophir Y, Golding B: Recombinant human alpha-1 proteinase inhibitor: towards therapeutic use. Amino Acids. 2006 Jun;30(4):317-32. Epub 2006 May 26. [Article]
- External Links
- UniProt
- P01009
- Genbank
- K01396
- PubChem Substance
- 46506364
- 535
- Therapeutic Targets Database
- DAP001102
- PharmGKB
- PA10308
- RxList
- RxList Drug Page
- Wikipedia
- Alpha-1_antitrypsin
- FDA label
- Download (101 KB)
Clinical Trials
- Clinical Trials
Phase Status Purpose Conditions Count 4 Active Not Recruiting Treatment Pancreatitis, Chronic; Diabetes; Transplant 1 4 Completed Treatment Alpha 1-proteinase Inhibitor Deficiency / Emphysema 2 4 Completed Treatment Alpha-1 Antitrypsin Deficiency (AATD) 1 4 Completed Treatment Alpha-1 Antitrypsin Deficiency (AATD) / Healthy Subjects (HS) 1 4 Terminated Treatment Alpha-1 Antitrypsin Deficiency (AATD) 1 4 Withdrawn Treatment Alpha-1 Antitrypsin Deficiency (AATD) / Chronic Obstructive Pulmonary Disease (COPD) 1 3 Completed Treatment Alpha-1 Antitrypsin Deficiency (AATD) 3 3 Enrolling by Invitation Treatment Pulmonary Emphysema in Alpha-1 Antitrypsin Deficiency 1 3 Recruiting Treatment Alpha-1 Antitrypsin Deficiency (AATD) 1 3 Recruiting Treatment Pulmonary Emphysema in Alpha-1 PI Deficiency 1
Pharmacoeconomics
- Manufacturers
- Not Available
- Packagers
- Baxter International Inc.
- CSL Behring LLC
- Talecris Biotherapeutics
- Dosage Forms
Form Route Strength Powder, for solution Intravenous 1 g / vial Powder, for solution Intravenous 500 mg / vial Kit Intravenous 16 mg/1mL Injection, solution Intravenous 1 g/50mL Injection, powder, for solution Parenteral Injection Intravenous Powder, for solution Intravenous 25 mg / mL Powder, for solution Intravenous 1000 mg / vial Injection, solution Intravenous 1000 mg/20mL Solution Intravenous 1000 mg / 20 mL Injection, powder, lyophilized, for solution Intravenous 1000 mg Injection, powder, for solution Intravenous 4000 mg Injection, powder, for solution Intravenous 5000 mg Powder, for solution Intravenous 1000 MG Kit Intravenous 1000 mg/20mL Kit Intravenous 4000 mg/76mL Kit Intravenous 5000 mg/95mL Kit; powder, for solution Intravenous 1000 mg / vial Kit; powder, for solution Intravenous 4000 mg / vial Kit; powder, for solution Intravenous 5000 mg / vial Injection, powder, for solution Intravenous 1000 mg - Prices
Unit description Cost Unit Aralast 1000 mg vial 0.52USD vial Aralast np 1000 mg vial 0.52USD vial Aralast np 500 mg vial 0.52USD vial Aralast 500 mg vial 0.5USD vial Zemaira 1000 mg vial 0.5USD vial Prolastin 1000 mg vial 0.46USD vial Prolastin 500 mg vial 0.46USD vial Prolastin c 1000 mg vial 0.46USD vial DrugBank does not sell nor buy drugs. Pricing information is supplied for informational purposes only.- Patents
- Not Available
Properties
- State
- Liquid
- Experimental Properties
Property Value Source melting point (°C) 59 °C at pH 7.8 Tew D.J., Bottomley, S.P. J. Mol. Biol. 313:1161-1169 (2001) hydrophobicity -0.302 Not Available isoelectric point 5.37 Not Available
Targets
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- Kind
- Protein
- Organism
- Humans
- Pharmacological action
- Yes
- Actions
- Inhibitor
- General Function
- Serine-type endopeptidase activity
- Specific Function
- Modifies the functions of natural killer cells, monocytes and granulocytes. Inhibits C5a-dependent neutrophil enzyme release and chemotaxis.
- Gene Name
- ELANE
- Uniprot ID
- P08246
- Uniprot Name
- Neutrophil elastase
- Molecular Weight
- 28517.81 Da
References
- Topic A, Ljujic M, Nikolic A, Petrovic-Stanojevic N, Dopudja-Pantic V, Mitic-Milikic M, Radojkovic D: Alpha-1-antitrypsin phenotypes and neutrophil elastase gene promoter polymorphisms in lung cancer. Pathol Oncol Res. 2011 Mar;17(1):75-80. doi: 10.1007/s12253-010-9283-5. Epub 2010 Jun 3. [Article]
- Karnaukhova E, Ophir Y, Golding B: Recombinant human alpha-1 proteinase inhibitor: towards therapeutic use. Amino Acids. 2006 Jun;30(4):317-32. Epub 2006 May 26. [Article]
- Chen X, Ji ZL, Chen YZ: TTD: Therapeutic Target Database. Nucleic Acids Res. 2002 Jan 1;30(1):412-5. [Article]
Drug created on June 13, 2005 13:24 / Updated on September 16, 2021 13:04