Long-term Efficacy, Safety and Tolerability of Iptacopan in C3G or IC-MPGN

This is an open-label extension study to evaluate the long-term efficacy, safety and
tolerability of iptacopan in subjects with C3 glomerulopathy or idiopathic
immune-complex-membranoproliferative glomerulonephritis The primary purpose of this extension study is to collect long-term efficacy, safety and
tolerability data in eligible participants receiving open-label iptacopan after
completing treatment in the C3G Phase 2 proof of concept study CLNP023X2202.

The primary (at 9 months) and longer-term (>9 months) efficacy and safety data of
iptacopan collected from CLNP023X2202 participants will be used to support health
authority submissions.

This umbrella protocol will also allow:

- continued access to iptacopan to patients enrolled in the ongoing Phase 3 programs
(C3G and IC-MPGN)

- C3G study (CLNP023B12301): adults and adolescents

- IC-MPGN study (CLNP023B12302): adults and adolescents

- provision of additional efficacy and safety information following longer-term
treatment in C3G and IC-MPGN populations to support health authority submissions.

Efficacy and safety assessments at the 9 month visit of this extension study in
combination with data from CLNP023X2202 (baseline plus 3 months of treatment) allowed
evaluation of the effects of iptacopan on potential endpoint(s) at 12 months of iptacopan
treatment in C3G participants. The enrollment of C3G and IC-MPGN participants (adults and
adolescents) from Phase 3 studies, CLNP023B12301 and CLNP023B12302, permits longer-term
evaluation of the persistence of effects observed after iptacopan treatment up to 12
months. These longer term efficacy assessments may be compared to historical/concurrent
control data available from relevant real world databases in C3G or IC-MPGN patients and
used as supportive information for registration purposes.

This extension study is expected to continue until the drug product becomes commercially
available and accessible (anticipated to be up to approximately 66 months), or the
benefit-risk profile is no longer positive, or the program is discontinued for business
or strategic reasons.

"Baseline" refers to the Day 1 visit (pre-dose) of CLNP023X2202, CLNP023B12301 or
CLNP023B12302, whereas the Day 1 visit for this C3G/IC-MPGN extension study
(CLNP023B12001B) is identified as "Extension Day 1".