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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Associated Conditions

Emphysema

Associated Therapies

Contraindications & Blackbox Warnings

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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	inhibitor	Humans

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

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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
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Albutrepenonacog 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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Food Interactions

No interactions found.

Products

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International/Other Brands

Aralast (Baxter) / Prolastin (Talecris Biotherapeutics C formerly Bayer)

Brand Name Prescription Products

Name	Dosage	Strength	Route	Labeller	Marketing Start	Marketing End
Alpha1-proteinase Inhibitor (human)	Powder, for solution	1 g / vial	Intravenous	Grifols Therapeutics Llc	Not applicable	Not applicable
Alpha1-proteinase Inhibitor (human)	Powder, for solution	500 mg / vial	Intravenous	Grifols Therapeutics Llc	Not applicable	Not applicable
Aralast NP	Kit	16 mg/1mL	Intravenous	Baxter Laboratories	2010-09-01	2014-11-09
Aralast NP	Kit	16 mg/1mL	Intravenous	Baxter Laboratories	2010-09-01	2014-11-09
Aralast NP	Injection, powder, lyophilized, for solution; Kit	16 mg/1mL	Intravenous	Baxalta u.s. Inc.	2002-12-23	Not applicable
Aralast NP	Kit	16 mg/1mL	Intravenous	Baxter Laboratories	2010-04-19	2014-11-09
Aralast NP	Injection, powder, lyophilized, for solution; Kit	16 mg/1mL	Intravenous	Baxalta u.s. Inc.	2002-12-23	Not applicable
Glassia	Injection, solution	1 g/50mL	Intravenous	Baxalta US Inc.	2010-07-01	Not applicable
Glassia	Solution	1000 mg / 50 mL	Intravenous	Takeda	Not applicable	Not applicable
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

Mixture Products

Name	Ingredients	Dosage	Route	Labeller	Marketing Start	Marketing End	Region	Image
Prolastin-C	Alpha-1-proteinase inhibitor (1000 mg/20mL) + Water (1 mL/1mL)	Injection, powder, lyophilized, for solution; Kit	Intravenous	GRIFOLS USA, LLC	1987-12-02	Not applicable

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]
FDA Approved Products: PROLASTIN® 5 -C LIQUID (Alpha1-Proteinase Inhibitor [Human]) Solution for Intravenous Injection [Link]

External Links

UniProt: P01009
Genbank: K01396
PubChem Substance: 46506364
RxNav: 535
Therapeutic Targets Database: DAP001102
PharmGKB: PA10308
RxList: RxList Drug Page
Wikipedia: Alpha-1_antitrypsin

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	Not Yet Recruiting	Treatment	Alpha-1 Antitrypsin Deficiency (AATD) / Chronic Obstructive Pulmonary Disease (COPD)	1
4	Not Yet Recruiting	Treatment	Bronchiectasis Adult	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

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
Injection, powder, lyophilized, for solution; kit	Intravenous	16 mg/1mL
Kit	Intravenous	16 mg/1mL
Injection, solution	Intravenous	1 g/50mL
Solution	Intravenous	1000 mg / 50 mL
Injection, powder, for solution	Parenteral
Injection	Intravenous	1000 mg
Powder, for solution	Intravenous	25 mg / mL
Injection, powder, lyophilized, for solution; kit	Intravenous
Injection; kit	Intravenous	1000 mg/20mL
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
Injection, powder, lyophilized, for solution; kit	Intravenous	1000 mg/20mL
Injection, powder, lyophilized, for solution; kit	Intravenous	4000 mg/76mL
Injection, powder, lyophilized, for solution; 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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Details1. Neutrophil elastase

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 at June 13, 2005 13:24 / Updated at January 24, 2023 16:16

Alpha-1-proteinase inhibitor

Identification

Pharmacology

Interactions

Products

Categories

Chemical Identifiers

References

Clinical Trials

Pharmacoeconomics

Properties

Targets

References