ALDOSE REDUCTASE INHIBITORS FOR TREATING SORBITOL DEHYDROGENASE DEFICIENCY
The disclosure relates to methods for a genetic and/or metabolic disorder that alters sorbitol metabolism or causes over production of sorbitol, such as SDH deficiency, hereditary neuropathy using aldose reductase inhibitors.
1 - 21 . (canceled)
22 . A method of treating hereditary neuropathy associated with sorbitol-dehydrogenase (SDH) deficiency comprising administering a therapeutically effective amount of an aldose reductase inhibitor to a subject in need thereof, wherein the aldose reductase inhibitor is
or a salt thereof.
23 . The method of claim 22 , wherein the effective amount of the aldose reductase inhibitor is sufficient to reduce sorbitol accumulation in the subject.
24 - 26 . (canceled)
27 . The method of claim 22 , wherein the hereditary neuropathy associated with SDH deficiency is distal hereditary motor neuropathy (dHMN).
28 . The method of claim 22 , wherein the hereditary neuropathy associated by with SDH deficiency is Charcot-Marie-Tooth (CMT) disease.
29 . The method of claim 28 , wherein the CMT disease is CMT neuropathy type 1 disease (CMT-1).
30 . The method of claim 28 , wherein the CMT disease is CMT neuropathy type 2 (CMT-2).
31 - 37 . (canceled)
38 . The method of claim 22 , wherein the subject is a human.
39 . The method of claim 38 , wherein the subject has diabetes.
40 . The method of claim 39 , wherein the subject has a complication of diabetes.
41 - 51 . (canceled)
52 . A method of treating hereditary neuropathy associated with sorbitol-dehydrogenase (SDH) deficiency, comprising administering a therapeutically effective amount of an aldose reductase inhibitor to a subject in need thereof, wherein the aldose reductase
53 . The method of claim 52 , wherein the effective amount of the aldose reductase inhibitor is sufficient to reduce sorbitol accumulation in the subject.
54 . The method of claim 52 , wherein the hereditary neuropathy associated with SDH deficiency is distal hereditary motor neuropathy (dHMN).
55 . The method of claim 52 , wherein the hereditary neuropathy associated with SDH deficiency is Charcot-Marie-Tooth (CMT) disease.
56 . The method of claim 55 , wherein the CMT disease is CMT neuropathy type 1 (CMT-1).
57 . The method of claim 55 , wherein the CMT disease is CMT neuropathy type 2 (CMT-2).
58 . The method of claim 52 , wherein the subject is a human.
59 . The method of claim 58 , wherein the subject has diabetes.
60 . The method of claim 59 , wherein the subject has a complication of diabetes.
61 . The method of claim 22 , wherein the aldose reductase inhibitor is administered orally once a day.
62 . The method of claim 52 , wherein the aldose reductase inhibitor is administered orally once a day.