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sepofarsen

Phase 2

Leber Congenital Amaurosis 10 | Small molecule | Rare Disease |ProQR Therapeutics N.V.|Last Updated: Mar 17, 2022

Target and mechanism

Molecular targetCEP290
Target classPositive Modulator
ModalitySmall molecule

Success Probability

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Market & Valuation

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Trial Design

RandomizedDouble-BlindACTIVE_CONTROLLEDDMC
Total Trials1
Total Enrollment36

FDA Designations

No designations recorded

Clinical trial landscape

sepofarsen · 1 trial · 10 indications

Phase 2 1
NCT03913143A Study to Evaluate Efficacy, Safety, Tolerability and Exposure After a Repeat-dose of Sepofarsen (QR-110) in LCA10 (ILLUMINATE)Leber Congenital Amaurosis 10
ACTIVE NOT_RECRUITING36 Analytics
PHASE2ACTIVE NOT_RECRUITING
A Study to Evaluate Efficacy, Safety, Tolerability and Exposure After a Repeat-dose of Sepofarsen (QR-110) in LCA10 (ILLUMINATE)
Leber Congenital Amaurosis 10Unlock trial analytics

Study Endpoints

Primary Endpoints

Change in BCVA
12 months

Change in Best-corrected visual acuity (BCVA) relative to baseline after 12 months of treatment versus sham-procedure

Secondary Endpoints

Change from baseline in BCVA ≤ -0.3 LogMAR
12 and 24 months
Clinical meaningful improvement in subjects with BCVA ≤ 1.7 LogMAR
12 and 24 months
Change in BCVA based on FrACT
12 and 24 months
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Study Design & Arms

AllocationRANDOMIZED
MaskingQUADRUPLE
ModelPARALLEL
PurposeTREATMENT

Treatment Arms

ArmTypeDescription
Group 1: Dose 1 sepofarsen (QR-110)EXPERIMENTALInitial loading dose, followed by maintenance doses at month 3 and every 6 months there after, administered by intravitreal injection (24 months duration of treatment). After 12 months treatment of the contralateral eye may be initiated
Group 2: Dose 2 sepofarsen (QR-110)ACTIVE_COMPARATORInitial loading dose, followed by maintenance doses at month 3 and every 6 months there after, administered by intravitreal injection (24 months duration of treatment). After 12 months treatment of the contralateral eye may be initiated
Group 3: ShamSHAM_COMPARATORSham procedure (no experimental drug administered), Day 1, month 3 and every six months there after. After 12 months cross over to active study drug may be initiated

Interventions

NameTypeDescription
sepofarsenDRUGRNA antisense oligonucleotide for intravitreal injection
ShamOTHERSham-Procedure (no experimental drug administered)
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Eligibility Criteria

Age Range8 Years to N/A
SexALL
Healthy VolunteersNo
Study Sites14

Main Inclusion Criteria Relating to Study Initiation: * Male or female, ≥ 8 years of age at Screening with a clinical diagnosis of LCA10 and a molecular diagnosis of homozygosity or compound heterozygosity for the c.2991+1655A\>G mutation, based on genotyping analysis at Screening. A historic genot...

Countries:United StatesBelgiumBrazilCanadaFranceGermanyItalyNetherlandsUnited Kingdom
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Frequently asked questions about sepofarsen

What is sepofarsen used for?

Sepofarsen is an investigational small molecule being developed for Leber Congenital Amaurosis 10 (LCA10), a rare inherited retinal disease that causes severe vision loss and blindness. It is designed to address the underlying genetic cause of the condition. Sepofarsen is currently in Phase 2 clinical development.

How does sepofarsen work?

Sepofarsen is an antisense oligonucleotide (ASO), a class of small molecules that target RNA to modulate gene expression. It is designed to correct the splicing defect associated with Leber Congenital Amaurosis 10, potentially restoring functional protein production. This mechanism aims to address the root cause of the disease.

Who is developing sepofarsen?

Sepofarsen is being developed by ProQR Therapeutics N.V., a biopharmaceutical company focused on RNA-based therapies. ProQR Therapeutics is publicly traded under the ticker symbol PRQR. The company is advancing sepofarsen through clinical trials for Leber Congenital Amaurosis 10.

What phase is sepofarsen in?

Sepofarsen is in Phase 2 clinical development. It is an investigational drug, meaning it has not been approved by regulatory authorities and is still being studied for safety and efficacy. The ongoing Phase 2 trial is evaluating the drug in patients with Leber Congenital Amaurosis 10.

What clinical trials is sepofarsen in?

Sepofarsen is being studied in a Phase 2 clinical trial with the identifier NCT03913143, titled "A Study to Evaluate Efficacy, Safety, Tolerability and Exposure After a Repeat-dose of Sepofarsen (QR-110) in LCA10 (ILLUMINATE)." The trial is active but not recruiting, with an enrollment of 36 participants across multiple countries.

Is sepofarsen the same as QR-110?

Yes, sepofarsen is also known as QR-110. The clinical trial NCT03913143 refers to sepofarsen as QR-110 in its title. Both names refer to the same investigational drug being developed by ProQR Therapeutics for Leber Congenital Amaurosis 10.