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Identification
NameAlpha-1-proteinase inhibitor
Accession NumberDB00058  (BIOD00002, BTD00002)
TypeBiotech
GroupsApproved
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.

Protein structureDb00058
Protein chemical formulaC2001H3130N514O601S10
Protein average weight44324.5000
Sequences
>DB00058 sequence
EDPQGDAAQKTDTSHHDQDHPTFNKITPNLAEFAFSLYRQLAHQSNSTNIFFSPVSIATA
FAMLSLGTKADTHDEILEGLNFNLTEIPEAQIHEGFQELLRTLNQPDSQLQLTTGNGLFL
SEGLKLVDKFLEDVKKLYHSEAFTVNFGDTEEAKKQINDYVEKGTQGKIVDLVKELDRDT
VFALVNYIFFKGKWERPFEVKDTEEEDFHVDQVTTVKVPMMKRLGMFNIQHCKKLSSWVL
LMKYLGNATAIFFLPDEGKLQHLENELTHDIITKFLENEDRRSASLHLPKLSITGTYDLK
SVLGQLGITKVFSNGADLSGVTEEAPLKLSKAVHKAVLTIDEKGTEAAGAMFLEAIPMSI
PPEVKFNKPFVFLMIEQNTKSPLFMGKVVNPTQK
Download FASTA Format
Synonyms
SynonymLanguageCode
Alpha 1-antitrypsinNot AvailableNot Available
Alpha-1 protease inhibitorNot AvailableNot Available
Alpha-1-antiproteinaseNot AvailableNot Available
Alpha-1-antitrypsin precursorNot AvailableNot Available
SaltsNot Available
Brand names
NameCompany
AralastBaxter
ProlastinTalecris Biotherapeutics C formerly Bayer
Brand mixturesNot Available
Categories
CAS number9041-92-3
Taxonomy
KingdomOrganic Compounds
SuperclassOrganic Acids
ClassCarboxylic Acids and Derivatives
SubclassAmino Acids, Peptides, and Analogues
Direct parentPeptides
Alternative parentsNot Available
SubstituentsNot Available
Classification descriptionNot Available
Pharmacology
IndicationFor treatment of panacinar emphysema
PharmacodynamicsPrevents excessive accumulation of active neutrophil elastase and consequent proteolysis of elastin tissues in alveolar lung structures. This prevents the development of emphysema.
Mechanism of actionAlpha-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.
AbsorptionNot Available
Volume of distribution
  • 5632 ± 2006 mL [ARALAST NP]
  • 5618 ± 1618 mL [Aralast]
Protein bindingNot Available
Metabolism
Route of eliminationNot Available
Half lifeNot Available
Clearance
  • 940 +/- 275 mL/day [Patients with congenital α1-PI deficiency with single IV infusion of 60 mg/kg]
ToxicityNot Available
Affected organisms
  • Humans and other mammals
PathwaysNot Available
SNP Mediated EffectsNot Available
SNP Mediated Adverse Drug ReactionsNot Available
Pharmacoeconomics
ManufacturersNot Available
Packagers
Dosage forms
FormRouteStrength
Injection, powder, lyophilized, for solutionIntravenous
Prices
Unit descriptionCostUnit
Aralast 1000 mg vial0.52USDvial
Aralast np 1000 mg vial0.52USDvial
Aralast np 500 mg vial0.52USDvial
Aralast 500 mg vial0.5USDvial
Zemaira 1000 mg vial0.5USDvial
Prolastin 1000 mg vial0.46USDvial
Prolastin 500 mg vial0.46USDvial
Prolastin c 1000 mg vial0.46USDvial
DrugBank does not sell nor buy drugs. Pricing information is supplied for informational purposes only.
PatentsNot Available
Properties
Stateliquid
Experimental Properties
PropertyValueSource
melting point59 °C at pH 7.8Tew D.J., Bottomley, S.P. J. Mol. Biol. 313:1161-1169 (2001)
hydrophobicity-0.302Not Available
isoelectric point5.37Not Available
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 Reference
  1. 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. Pubmed
External Links
ResourceLink
UniProtP01009
GenbankK01396
PharmGKBPA10308
Drug Product Database2204606
RxListhttp://www.rxlist.com/cgi/generic3/zemaira.htm
WikipediaAlpha_1-antitrypsin
ATC CodesNot Available
AHFS CodesNot Available
PDB Entries
FDA labelNot Available
MSDSNot Available
Interactions
Drug InteractionsNot Available
Food InteractionsNot Available

Targets

1. Neutrophil elastase

Kind: protein

Organism: Human

Pharmacological action: yes

Actions: inhibitor

Components

Name UniProt ID Details
Neutrophil elastase P08246 Details

References:

  1. 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. 2010 Jun 3. Pubmed
  2. 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. Pubmed
  3. Chen X, Ji ZL, Chen YZ: TTD: Therapeutic Target Database. Nucleic Acids Res. 2002 Jan 1;30(1):412-5. Pubmed

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Drug created on June 13, 2005 07:24 / Updated on September 13, 2013 10:48