Alpha-1-proteinase inhibitor
Also known as: A1PI, AAT, Alpha-1-antitrypsin, Alpha‑1 antitrypsin, Glassia, Prolastin, Prolastin-C, SERPINA1, Zemaira
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Summary
Alpha-1-proteinase inhibitor (alpha‑1 antitrypsin) is a plasma‑derived or recombinant serpin used as replacement therapy for individuals with hereditary A1AT deficiency to slow emphysema progression.
Mechanism of Action
Inhibits neutrophil elastase and other serine proteases, protecting lung tissue from proteolytic damage.
Routes of Administration
Goals & Uses
- Reduction of acute exacerbationsPulmonaryLow
- Slowing emphysema progression in alpha-1-antitrypsin deficiencyPulmonary / Disease ModificationModerate
- Raising serum A1PI above protective thresholdBiochemical/Surrogate EndpointHigh
- Augmentation therapy for A1AT deficiencyPulmonary DiseaseHigh
- Prevention of liver disease progressionHepatic DiseaseModerate
Contraindications
- IgA deficiency with anti-IgA antibodiesImmunologicalHigh
- Hypersensitivity to alpha‑1 antitrypsin productAllergyHigh
- Hypersensitivity to A1PI or any product excipientImmunologicalHigh
- Severe liver dysfunctionHepaticModerate
- Alpha-1-antitrypsin deficiency without emphysema (Pi*MZ heterozygotes with normal levels)Indication MismatchModerate
Adverse Effects
- AnaphylaxisImmunologicRareSevere life-threatening allergic reaction
- HeadacheNeurologicCommonPain in the head or upper neck
- Upper respiratory tract infectionInfectiousCommon
- Theoretical risk of transmissible pathogensInfectiousRare
- Infusion-related reactionsHypersensitivityCommon
- Infusion-related reactions (fever, chills, flushing)Immunological / InfusionUncommon
- DizzinessNeurologicUncommonFeeling faint, lightheaded, or unsteady
Drug Interactions
- Anticoagulants (e.g., warfarin)Moderate
- Tobacco smoke / cigarette smokingHigh
Population Constraints
- PregnancyReproductive SafetyRelative
- Pediatric patientsAgeRelative
- Patients with cardiac or renal impairmentComorbidityRelative
- Pediatric patients (<12 years)PediatricRelative
- Pregnant womenReproductiveRelative
Regulatory Status
- European UnionApprovedApproved: Alpha‑1 antitrypsin deficiencyEMA-approved plasma‑derived and recombinant forms.
- United StatesApprovedApproved: Alpha‑1 antitrypsin deficiency (augmentation therapy)FDA-approved products include Prolastin, Zemaira, Glassia.
- United KingdomApprovedApproved: Alpha‑1 antitrypsin deficiencyMHRA-approved for augmentation therapy.
FDA‑approved (e.g., Prolastin, Zemaira, Glassia) for augmentation therapy in A1AT deficiency; also approved in EU and UK.
Evidence & Sources
No sources recorded yet.
Frequently Asked Questions
What is Alpha-1-proteinase inhibitor?
Alpha-1-proteinase inhibitor (alpha‑1 antitrypsin) is a plasma‑derived or recombinant serpin used as replacement therapy for individuals with hereditary A1AT deficiency to slow emphysema progression.
What is Alpha-1-proteinase inhibitor used for?
Alpha-1-proteinase inhibitor is educationally associated with: Reduction of acute exacerbations, Slowing emphysema progression in alpha-1-antitrypsin deficiency, Raising serum A1PI above protective threshold, Augmentation therapy for A1AT deficiency, Prevention of liver disease progression. Educational only — not medical advice.
How is Alpha-1-proteinase inhibitor administered?
Recorded routes of administration: Intravenous.
What are the potential side effects of Alpha-1-proteinase inhibitor?
Reported adverse effects include: Anaphylaxis, Headache, Upper respiratory tract infection, Theoretical risk of transmissible pathogens, Infusion-related reactions, Infusion-related reactions (fever, chills, flushing), Dizziness. This list is not exhaustive — consult a qualified clinician.
Who should avoid Alpha-1-proteinase inhibitor?
Recorded contraindications: IgA deficiency with anti-IgA antibodies, Hypersensitivity to alpha‑1 antitrypsin product, Hypersensitivity to A1PI or any product excipient, Severe liver dysfunction, Alpha-1-antitrypsin deficiency without emphysema (Pi*MZ heterozygotes with normal levels). Consult a qualified clinician before use.