A NEW TREATMENT FOR FACIO-SCABORO-SHOULDER MUSCULAR DYSTROPHY (FSHD)

Claims

1. An antibody to myostatin for use in the treatment, prevention, slowing the progression of and / or alleviating facioscapulohumeral muscular dystrophy (FSHD).

2. An antibody to myostatin for use according to claim 1, wherein the antibody to myostatin binds to latent myostatin and does not bind to mature myostatin, wherein the antibody blocks non-proteolytic spontaneous release of mature myostatin from latent myostatin and inhibits activation of myostatin.

3. An antibody to myostatin for use according to claim 1 or 2, wherein the antibody to myostatin comprises six complementarity determining regions (CDRs): CDRH1, CDRH2, CDRH3, CDRL1, CDRL2 and CDRL3, wherein CDRH1 comprises the sequence presented in SEQ ID 1, CDRH2 comprises the sequence presented in SEQ ID 2, CDRH3 comprises the sequence presented in SEQ ID 3, CDRL1 comprises the sequence presented in SEQ ID 4, CDRL2 comprises the sequence presented in SEQ ID 5 and CDRL3 comprises the sequence presented in SEQ ID 6.

4. An antibody to myostatin for use according to claims 1-3, wherein the antibody to myostatin comprises a VH chain comprising the amino acid sequence of SEQ ID 7 and a VL chain comprising the amino acid sequence of SEQ ID 8.

5. An antibody to myostatin for use according to claim 14, wherein the antibody to myostatin comprises a heavy chain comprising the amino acid sequence of SEQ ID 9 and a light chain comprising the amino acid sequence of SEQ ID 10.

6. An antibody to myostatin for use according to claims 1-5 in a patient (in particular, a patient in need thereof), in particular, wherein the patient is a human (for example, a male or female human).

7. An antibody to myostatin for use according to claims 1-6, wherein the antibody to myostatin is administered to a subject, preferably a human subject, every four weeks at a dose of 90 mg.

8. An antibody to myostatin for use according to claims 1-7, wherein the antibody to myostatin is administered subcutaneously.