Use of Hsp70 as a regulator of enzymatic activity
The present invention concerns a method for modulating the enzymatic activity of an enzyme, wherein said enzyme interacts with BMP, said method comprising the step of administering or inducing Hsp70, or a functional fragment or variant thereof, in a form suitable for allowing interaction between BMP and Hsp70, or said functional fragment or variant thereof, and thereby modulating the enzymatic activity of an enzyme interacting with BMP.
1. A method for treating Niemann-Pick disease Type C (NPC) in a human comprising orally administering arimoclomol, or a pharmaceutically acceptable salt thereof, to the human.
2. The method of claim 1 , wherein the human is orally administered a daily dosage of arimoclomol, or a pharmaceutically acceptable salt thereof, of from about 1 ug to about 100 mg per kilogram of body weight.
3. The method of claim 2 , further comprising administering Miglustat to the human.
4. The method of claim 2 , further comprising administering enzyme replacement therapy to the human.
5. The method of claim 4 , wherein the enzyme replacement therapy is imiglucerase, agalsidase beta, or agalsidase alpha.