Long Term Extension Trial of Setmelanotide

The purpose of this protocol is to continue the assessment of setmelanotide treatment in
patients who have successfully completed all critical study evaluations in a prior trial
(index protocol) of setmelanotide for the treatment of obesity associated with genetic
defects in the leptin-melanocortin pathway upstream of the MC4 receptor. The primary
objectives of this extension trial are to explore the long-term safety and tolerability of
setmelanotide for up to 5 years or until drug is otherwise available through authorized use.
Patients can enter this protocol immediately upon completion of their index protocol such
that dosing of setmelanotide continues without gaps in therapy.

Inclusion Criteria:

1. Completed participation (all critical study evaluations) on active drug in a previous
setmelanotide study for obesity associated with genetic defects upstream of the MC4
receptor in the leptin-melanocortin pathway.

2. Study participant and/or parent or guardian is able to communicate well with the
investigator, to understand and comply with the requirements of the study, and be able
to understand and sign the written informed consent/assent.

3. Female participants of child-bearing potential must agree to use contraception as
outlined in the protocol. Female participants of non-childbearing potential, defined
as surgically sterile (status post hysterectomy, bilateral oophorectomy, or bilateral
tubal ligation) or post-menopausal for at least 12 months (and confirmed with a FSH
level in the post-menopausal lab range), do not require contraception during the
study.

4. Male participants with female partners of childbearing potential must agree to a
double barrier method if they become sexually active during the study or the extension
phase. Male patients must not donate sperm during and for 90 days following their
participation in the study.

Exclusion Criteria:

1. Current, clinically significant disease, if severe enough to interfere with the study
and/or would confound the results. Any such patients should be discussed with the sponsor
prior to inclusion.