alpha-1-antitrypsin; alpha-1 protease inhibitor; alpha-1-antiproteinase; Serpin A1 (SERPINA1, AAT, PI, PRO0684, PRO2209)

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Function

Structure

Compartment

Alternative splicing

Expression

plasma

Pathology

Polymorphism

  • M1V 44-49% (most common allele)
  • M1A 20-23%
  • M2 10=11%;
  • M3 14-19%

Pharmacology

Available only through specialty pharmacies

Tradename: Prolastin, Zemaira, Aralast

Indications

alpha-1 antitrypsin deficiency

Dosage

  • 60 mg/kg IV once weekly

Injection: > 20 mg alpha-1 antitrypsin/mL (vials 500 & 1000 mg)

Adverse effects

More general terms

More specific terms

Additional terms

References

  1. Fundamentals of Clinical Chemistry 3rd ed., N.W. Teitz ed., W.B. Saunders, 1988, pg 330
  2. Kaiser Permanente Northern California Regional Drug Formulary, 1998
  3. Prescriber's Letter 11(2):suppl 2004
  4. UniProt http://www.uniprot.org/uniprot/P01009.html
  5. GeneReviews http://www.ncbi.nlm.nih.gov/sites/genetests/lab/gene/SERPINA1
  6. Wikipedia; Note: alpha-1 antitrypsin entry http://en.wikipedia.org/wiki/alpha_1-antitrypsin

Database