Treatment of amyotrophic lateral sclerosis

A compound of the formula:

• or a pharmaceutically acceptable salt thereof, wherein:
• p is 1-2;
• designates a single or double bond, wherein one is a single bond and one is a double bond;
• each R1 is independently —R, —OR, —SR, —S(O)R, —SO2R, —OSO2R, —N(R)2, —CN, —NO2—NRC(O)R, —NRC(O)(CO)R, —NRC(O)N(R)2, —NRC(O)OR, —N(R)S(O)R, —N(R)SO2R, —N(R)SO2OR, —C(O)R, —C(O)OR, —OC(O)R, —OC(O)OR, —C(O)N(R)2, —OC(O)N(R)2, or an optionally substituted 3-8 membered saturated, partially unsaturated, or aryl monocyclic ring having 0-4 heteroatoms independently selected from nitrogen, oxygen, or sulfur, or an optionally substituted 8-10 membered saturated, partially unsaturated, or aryl bicyclic ring having 0-4 heteroatoms independently selected from nitrogen, oxygen, or sulfur;
• or
• two R1 groups are taken together to form
• wherein:
• X is N or C;
• each R5 is independently —R, —OR, —SR, —S(O)R, —SO2R, —OSO2R, —N(R)2, —CN, —NO2—NRC(O)R, —NRC(O)(CO)R, —NRC(O)N(R)2, —NRC(O)OR, —N(R)S(O)R, —N(R)SO2R, —N(R)SO2OR, —C(O)R, —C(O)OR, —OC(O)R, —OC(O)OR, —C(O)N(R)2, —OC(O)N(R)2, or a 3-8 membered saturated, partially unsaturated, or aryl monocyclic ring having 0-4 heteroatoms independently selected from nitrogen, oxygen, or sulfur, or an 8-10 membered saturated, partially unsaturated, or aryl bicyclic ring having 0-4 heteroatoms independently selected from nitrogen, oxygen, or sulfur, wherein R5 is optionally substituted with 1-5 R groups;
• each R is independently hydrogen, halogen, optionally substituted C1-20 aliphatic, optionally substituted C1-20 heteroaliphatic, optionally substituted phenyl, or optionally substituted arylalkyl, or two R on the same nitrogen are taken together to form a 5-6 membered saturated, partially saturated, or aryl ring having 1-3 heteroatoms independently selected from nitrogen, oxygen, and sulfur, or two R on the same carbon or on adjacent carbons are optionally taken together to form a 3-6 membered saturated cycloalkyl or fused monocyclic ring containing 0-2 heteroatoms independently selected from nitrogen, oxygen, and sulfur;
• n is 0-1;
• each R2 and R3 are independently —R, —OR, —SR, —S(O)R, —SO2R, —OSO2R, —C(O)R, —C(O)OR, —OC(O)R, —OC(O)OR, —C(O)N(R)2, —OC(O)N(R)2, a suitable amino protecting group, or an optionally substituted 3-8 membered saturated, partially unsaturated, or aryl monocyclic ring having 0-4 heteroatoms independently selected from nitrogen, oxygen, or sulfur, or an optionally substituted 8-10 membered saturated, partially unsaturated, or aryl bicyclic ring having 0-4 heteroatoms independently selected from nitrogen, oxygen, or sulfur;
• R0 is R, —OR, —SR, —S(O)R, —SO2R, —OSO2R, N(R)2, —NRC(O)R, —NRC(O)(CO)R, —NRC(O)N(R)2, —NRC(O)OR, —N(R)S(O)R, —N(R)SO2R, —N(R)SO2OR, —C(O)R, —C(O)OR, —OC(O)R, —OC(O)OR, —C(O)N(R)2, —OC(O)N(R)2, or
• wherein:
• L is a valence bond or a bivalent saturated or partially unsaturated C1-10 hydrocarbon chain, wherein 1-4 methylene units of L are optionally and independently replaced by —O—, —S—, —C(O)—, —OC(O)—, —C(O)O—, —OC(O)O—, —S(O)—, —S(O)2—, —OSO2O—, —N(R)C(O)—, —C(O)NR—, —N(R)C(O)O—, —OC(O)NR—, —N(R)C(O)NR—, —N(R6)—, or -Cy-, and wherein L is optionally substituted with 1-4 R groups;
• wherein:
• each -Cy- is independently a bivalent optionally substituted saturated, partially unsaturated, or aromatic monocyclic or bicyclic ring selected from a 6-10 membered arylene, a 5-10 membered heteroarylene having 1-4 heteroatoms independently selected from oxygen, nitrogen, or sulfur, a 3-8 membered carbocyclylene, or a 3-10 membered heterocyclylene having 1-4 heteroatoms independently selected from oxygen, nitrogen, or sulfur;
• R6 is —R, —OR, —SR, —S(O)R, —SO2R, —OSO2R, —C(O)R, —C(O)OR, —OC(O)R, —OC(O)OR, —C(O)N(R)2, —OC(O)N(R)2, a suitable amino protecting group, or an optionally substituted 3-8 membered saturated, partially unsaturated, or aryl monocyclic ring having 0-4 heteroatoms independently selected from nitrogen, oxygen, or sulfur, or an optionally substituted 8-10 membered saturated, partially unsaturated, or aryl bicyclic ring having 0-4 heteroatoms independently selected from nitrogen, oxygen, or sulfur;
• Ring A is a 3-8 membered saturated, partially unsaturated, or aryl monocyclic ring optionally containing 0-4 heteroatoms independently selected from nitrogen, oxygen, or sulfur, or an 8-10 membered saturated, partially unsaturated, or aryl bicyclic ring optionally containing 0-4 heteroatoms independently selected from nitrogen, oxygen, or sulfur, and wherein Ring A is optionally substituted with m occurrences of R4;
• m is 0-5;
• and
• each R4 is independently —R, —OR, —SR, —CN, —S(O)R, —SO2R, —OSO2R, —N(R)2, —NO2, —NRC(O)R, —NRC(O)(CO)R, —NRC(O)N(R)2, —NRC(O)OR, —N(R)S(O)R, —N(R)SO2R, —N(R)SO2OR, —C(O)R, —C(O)OR, —OC(O)R, —OC(O)OR, —C(O)N(R)2, —OC(O)N(R)2, or a 3-8 membered saturated, partially unsaturated, or aryl monocyclic ring optionally containing 0-4 heteroatoms independently selected from nitrogen, oxygen, or sulfur.