Phenothiazine diaminium salts and their use
Disclosed are compounds of general formula (I): and pharmaceutically acceptable salts thereof, formulations, methods and uses in, for example, the treatment of disease.
1. A method of treating a disease of protein aggregation comprising administering to a subject in need thereof the compound of the following formula:
or a pharmaceutically acceptable salt, solvate, or hydrate thereof, wherein the protein is selected from TDP-43, Huntingtin, and α-synuclein.
2. The method of claim 1 , wherein the administering is oral.
3. The method of claim 2 , wherein the administering is selected from:
(a) about 100mg of compound, 3times daily;
(b) about 150mg of compound, 2times daily; and
(c) about 200mg of compound, 2times daily.
4. A method of treating a disease of protein aggregation comprising administering to a subject in need thereof the compound of the following formula:
or a pharmaceutically acceptable salt, solvate, or hydrate thereof, wherein the disease is selected from Huntington's disease, Parkinson's disease, amyotrophic lateral sclerosis, mild cognitive impairment (MCI) and frontotemporal lobar degeneration (FTLD-TDP).
5. The method of claim 4 , wherein the administering is oral.
6. The method of claim 5 , wherein the administering is selected from
(a) about 100mg of compound, 3times daily;
(b) about 150mg of compound, 2times daily; and
(c) about 200mg of compound, 2times daily.
7. The method of claim 4 , wherein the disease is Huntington's disease.
8. The method of claim 4 , wherein the disease is Parkinson's disease.
9. The method of claim 4 , wherein the disease is amyotrophic lateral sclerosis.
10. The method of claim 4 , wherein the disease is MCI.
11. The method of claim 4 , wherein the disease is FTLD-TDP.