COMPOUNDS AND THEIR USE FOR THE TREATMENT OF ALPHA1-ANTITRYPSIN DEFICIENCY
The invention relates to specified benzamide compounds of formula (1), and pharmaceutical compositions containing the compounds. The compounds may be inducers of α 1 -antitrypsin (A1AT), and may be used in the treatment of a disease or disorder such as α 1 -antitrypsin deficiency (A1AD or AATD).
1 . A compound represented by the structure:
or pharmaceutically acceptable salt of any one thereof.
2 . The compound of claim 1 , wherein the compound is in a crystalline form.
3 . A pharmaceutical composition comprising a compound represented by the structure:
or pharmaceutically acceptable salt of any one thereof, and a pharmaceutically acceptable carrier.
4 .- 5 . (canceled)
6 . A method of inducing Z A1AT secretion in a subject in need thereof comprising administering to the subject a compound represented by the structure:
or pharmaceutically acceptable salt of any one thereof.
7 .- 11 . (canceled)
12 . The compound of claim 1 , wherein the compound is represented by:
or a pharmaceutically acceptable salt of any one thereof.
13 . The compound of claim 12 , wherein the compound is represented by:
or a pharmaceutically acceptable salt of any one thereof.
14 . The compound of claim 13 , wherein the compound is represented by:
or a pharmaceutically acceptable salt thereof.
15 . The compound of claim 13 , wherein the compound is represented by:
or a pharmaceutically acceptable salt thereof.
16 . The compound of claim 13 , wherein the compound is represented by:
or a pharmaceutically acceptable salt thereof.
17 . The compound of claim 13 , wherein the compound is represented by:
or a pharmaceutically acceptable salt thereof.
18 . The pharmaceutical composition of claim 3 , wherein the compound is represented by:
or a pharmaceutically acceptable salt of any one thereof.
19 . The pharmaceutical composition of claim 18 , wherein the compound is represented by
or pharmaceutically acceptable salt of any one thereof.
20 . The method of claim 6 , wherein the compound is represented by:
or pharmaceutically acceptable salt of any one thereof.
21 . The method of claim 20 , wherein the compound is represented by:
or pharmaceutically acceptable salt of any one thereof.
22 . The method of claim 21 , wherein the compound is represented by:
or a pharmaceutically acceptable salt thereof.
23 . The method of claim 21 , wherein the compound is represented by:
or a pharmaceutically acceptable salt thereof.
24 . The method of claim 21 , wherein the compound is represented by:
or a pharmaceutically acceptable salt thereof.
25 . The method of claim 21 , wherein the compound is represented by:
or a pharmaceutically acceptable salt thereof.
26 . The method of claim 21 , wherein the compound is represented by:
or a pharmaceutically acceptable salt thereof.