Methods of treating copper metabolism-associated diseases or disorders

A method for treating a copper metabolism-associated disease or disorder in a subject, the method comprising:

• administering to the subject a therapeutically effective amount of bis-choline tetrathiomolybdate for at least 48 weeks, wherein the subject is at least 12 years old.

The method of claim 5, wherein the subject is at least 18 years old.

A method for sequestering copper in a subject, the method comprising:

• administering to the subject a therapeutically effective amount of bis-choline tetrathiomolybdate for at least 48 weeks, wherein the subject is at least 12 years old, and wherein the bis-choline tetrathiomolybdate sequesters copper in the subject by at least about 3.3-fold as measured by daily mean AUEC0-48W for dNCC and as compared to standard of care therapy.

A method for sequestering copper in a subject, the method comprising:

• administering to the subject a therapeutically effective amount of bis-choline tetrathiomolybdate for at least 48 weeks, wherein the subject is at least 12 years old and treatment-naïve or previously received standard of care therapy for ≤28 days, and wherein the bis-choline tetrathiomolybdate sequesters copper in the subject by at least about 4.9-fold as measured by daily mean AUEC0-48W for dNCC and as compared to standard of care therapy.

A method for sequestering copper in a subject, the method comprising:

• administering to the subject a therapeutically effective amount of bis-choline tetrathiomolybdate for at least 48 weeks, wherein the subject is at least 12 years old and previously received standard of care therapy for >28 days, and wherein the bis-choline tetrathiomolybdate sequesters copper in the subject by at least about 2.9-fold as measured by daily mean AUEC0-48W for dNCC and as compared to standard of care therapy.

The method of any one of claims 30 to 39, wherein, in the subject being administered bis-choline tetrathiomolybdate, the sequestering is higher (at least 10% higher, or at least 15% higher, or at least 20% higher, or at least 25% higher, or at least 30% higher, or at least 35% higher, or at least 40% higher, or at least 45% higher, or at least 50% higher) as compared to the sequestering in the subject when the subject receives standard of care treatment (as described in any of claims 79 to 83).

A method for mobilizing copper in a subject, the method comprising:

• administering to the subject a therapeutically effective amount of bis-choline tetrathiomolybdate for at least 48 weeks, wherein the subject is at least 12 years old, and wherein the bis-choline tetrathiomolybdate mobilizes copper in the subject by at least about 3.3-fold as measured by daily mean AUEC0-48W for dNCC and as compared to standard of care therapy.

A method for mobilizing copper in a subject, the method comprising:

• administering to the subject a therapeutically effective amount of bis-choline tetrathiomolybdate for at least 48 weeks, wherein the subject is at least 12 years old and treatment-naïve or previously received standard of care therapy for ≤28 days, and wherein the bis-choline tetrathiomolybdate mobilizes copper in the subject by at least about 4.9-fold as measured by daily mean AUEC0-48W for dNCC and as compared to standard of care therapy.

A method for mobilizing copper in a subject, the method comprising:

• administering to the subject a therapeutically effective amount of bis-choline tetrathiomolybdate for at least 48 weeks, wherein the subject is at least 12 years old and previously received standard of care therapy for >28 days, and wherein the bis-choline tetrathiomolybdate mobilizes copper in the subject by at least about 2.9-fold as measured by daily mean AUEC0-48W for dNCC and as compared to standard of care therapy.

A method for blocking absorption of copper in tissue of a subject, the method comprising:

• administering to the subject a therapeutically effective amount of bis-choline tetrathiomolybdate for at least 48 weeks, wherein the subject is at least 12 years old, wherein the therapeutically effective amount of bis-choline tetrathiomolybdate is sufficient to block absorption of copper in tissue of a subject.

A method for treating a copper metabolism-associated disease or disorder in a subject, the method comprising:

• blocking absorption of copper in tissue of the subject and sequestering copper in the subject by administering to the subject a therapeutically effective amount of bis-choline tetrathiomolybdate for at least 48 weeks, wherein the subject is at least 12 years old.

A method for treating a copper metabolism-associated disease or disorder in a subject, the method comprising:

• determining a concentration of total copper in the subject's plasma;
• and
• administering to the subject a therapeutically effective amount of bis-choline tetrathiomolybdate.