Alpha 1-antitrypsin deficiency. A conformational disease.

doi:10.1378/chest.110.6_supplement.243s

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Article

Alpha 1-antitrypsin deficiency. A conformational disease.

Chest - 1 Dec 1996

Carrell R W, Lomas D A, Sidhar S, Foreman R

Abstract excerpt

The serpin family of protease inhibitors, to which alpha 1-antitrypsin belongs, has the unique feature of a mobile reactive center. Mutations within the critical regions of the molecule that control this mobility can allow premature changes in conformation with consequent abnormalities in folding...

Topics

Humans
Liver Diseases
Models, Molecular
Mutation
Phenotype
Polymers
Protein Conformation
Serpins
Structure-Activity Relationship
alpha 1-Antitrypsin
alpha 1-Antitrypsin Deficiency

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