A1AT-1 (336-343) Heavy
Ref. 3D-CRB1301553
25nMol | 452.00 € |
Product Information
- H-AVL^TIDEK-OH
Alpha-1 anti-trypsin (A1AT) is the most abundant anti-protease in circulation. It is produced in the liver and is essential for regulating the activity of neutrophil elastase and proteinase-3 in the lung. In its native metastable state it has a five-stranded central β-sheet, it is then transformed into a thermodynamically favoured six-stranded central β-sheet conformation.Under normal conditions, A1AT inhibits non-specific destruction by the serine protease neutrophil elastase (NE), to avoid destruction of lung elastin and prevent damage to bronchial and alveolar wall integrity. Mutations in alpha-1 anti-trypsin are responsible for alpha-1 anti-trypsin deficiency (AATD), one of the most common hereditary disorders. In AATD, reduced AAT levels result in destruction of lung tissue by NE. AATD also leads to a predisposition to severe liver disease. The leucine residue at position 3 of this peptide is isotopically labelled with carbon-13 (6) and nitrogen-15 (1), giving this peptide a mass increase of 7 compared to the unlabelled peptide.
Chemical properties
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