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Inotersen

Phase 3

FAP | Small molecule | Rare Disease |Ionis Pharmaceuticals, Inc.|Last Updated: Dec 13, 2024

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Trial Design

RandomizedDouble-BlindACTIVE_CONTROLLEDDMC
Total Trials2
Total Enrollment308

FDA Designations

No designations recorded

Clinical trial landscape

Inotersen · 3 trials · 6 indications

Phase 3 2Phase 2 1
NCT04136184NEURO-TTRansform: A Study to Evaluate the Efficacy and Safety of Eplontersen (Formerly Known as ION-682884, IONIS-TTR-LRx and AKCEA-TTR-LRx) in Participants With Hereditary Transthyretin-Mediated Amyloid PolyneuropathyHereditary Transthyretin-Mediated Amyloid Polyneuropathy
COMPLETED168 Analytics
NCT02175004Extension Study Assessing Long Term Safety and Efficacy of IONIS-TTR Rx in Familial Amyloid Polyneuropathy (FAP)FAP
COMPLETED135 Analytics
PHASE3COMPLETED
NEURO-TTRansform: A Study to Evaluate the Efficacy and Safety of Eplontersen (Formerly Known as ION-682884, IONIS-TTR-LRx and AKCEA-TTR-LRx) in Participants With Hereditary Transthyretin-Mediated Amyloid Polyneuropathy
Hereditary Transthyretin-Mediated Amyloid PolyneuropathyUnlock trial analytics
PHASE3COMPLETED
Extension Study Assessing Long Term Safety and Efficacy of IONIS-TTR Rx in Familial Amyloid Polyneuropathy (FAP)
FAPUnlock trial analytics

Study Endpoints

Primary Endpoints

Change From Baseline in Modified Neuropathy Impairment Score Plus 7 (mNIS+7) at Week 66
Baseline, Week 66

mNIS+7 composite score is a measure of neurologic impairment evaluating muscle weakness, sensation, reflexes, nerve conduction, and autonomic function. mNIS+7 consists of 2 composite scores: the NIS composite score (maximum of 244 points) \& the modified +7 composite score (maximum of 102.32 points). mNIS+7 composite total score range= -22.32 to 346.32. Higher score indicated a lower function. Least square (LS) mean \& standard error (SE) were analyzed using Mixed Effects Model with Repeated Measures (MMRM). As pre-specified in the protocol, the efficacy of eplontersen was to be assessed by comparing participants enrolled in the eplontersen arm only with the external placebo group. There was no statistical comparison planned between the inotersen arm and the eplontersen-treated/external placebo group arms.

Change From Baseline in the Norfolk Quality of Life Diabetic Neuropathy (QoL-DN) Questionnaire at Week 66
Baseline, Week 66

The Norfolk QoL-DN score is a measure of physical function/large fiber neuropathy, symptoms, activities of daily living, small fiber neuropathy, and autonomic neuropathy. The Norfolk QoL-DN total score has a range of -4 to 138, and a higher score indicates poorer quality of life. LS mean and SE were analyzed using MMRM. As pre-specified in the protocol, the efficacy of eplontersen was to be assessed by comparing participants enrolled in the eplontersen arm only with the external placebo group. There was no statistical comparison planned between the inotersen arm and the eplontersen-treated/external placebo group arms.

Percent Change From Baseline in Serum TTR Concentration at Week 65
Baseline, Week 65

As pre-specified in the protocol, the efficacy of eplontersen was to be assessed by comparing participants enrolled in the eplontersen arm only with the external placebo group. There was no statistical comparison planned between the inotersen arm and the eplontersen-treated/external placebo group arms. Overall number analyzed is the number of participants with data available for analysis.

Percent Change From Baseline in Serum TTR Concentration at Week 35
Baseline, Week 35

As pre-specified in the protocol, the efficacy of eplontersen was to be assessed by comparing participants enrolled in the eplontersen arm only with the external placebo group. There was no statistical comparison planned between the inotersen arm and the eplontersen-treated/external placebo group arms. Overall number analyzed is the number of participants with data available for analysis.

Change From Baseline in Modified Neuropathy Impairment Score Plus 7 (mNIS+7) at Week 35
Baseline, Week 35

mNIS+7 composite score is a measure of neurologic impairment evaluating muscle weakness, sensation, reflexes, nerve conduction, and autonomic function. mNIS+7 consists of 2 composite scores: NIS composite score (maximum of 244 points) \& the modified +7 composite score (maximum of 102.32 points). mNIS+7 composite total score range= -22.32 to 346.32. Higher score indicated a lower function. LS mean and SE were analyzed using MMRM. As pre-specified in the protocol, the efficacy of eplontersen was to be assessed by comparing participants enrolled in the eplontersen arm only with the external placebo group. There was no statistical comparison planned between the inotersen arm and the eplontersen-treated/external placebo group arms.

Percentage of Participants With Treatment Emergent Adverse Events (TEAEs), Serious TEAEs, and TEAEs Related to Study Drug
From first dose of study drug up to 3 months post treatment period of 260 weeks (Up to approximately 272 weeks)

An adverse event (AE) is any unfavorable and unintended sign (including a clinically significant abnormal laboratory finding, for example), symptom, or disease temporally associated with the study or use of investigational drug product, whether or not the AE is considered related to the investigational drug product. A TEAE is defined as an adverse event with an onset that occurs after receiving study drug. An SAE is any untoward medical occurrence that at any dose that results in death, is life threatening, requires inpatient hospitalization or prolongation of existing hospitalization, results in persistent or significant disability/incapacity, leads to a congenital anomaly/birth defect, or is an important medical event. TEAEs considered related to the study drug as assessed by the Investigator are reported.

Percentage of Participants With Change From Baseline in Vital Signs
From first dose of study drug up to 3 months post treatment period of 260 weeks (Up to approximately 272 weeks)

Vital signs included blood pressure, heart rate, respiratory rate, and temperature. Only categories with at least one participant with event are reported.

Percentage of Participants With Change From Baseline in Weight
From first dose of study drug up to 3 months post treatment period of 260 weeks (Up to approximately 272 weeks)

As prespecified in the protocol, percentage of participants with change from baseline in weight is reported in 2 categories, decrease of ≥7% from Baseline and increase of ≥7% from Baseline.

Percentage of Participants With Clinically Significant Change From Baseline in Laboratory Test Values
From first dose of study drug up to 3 months post treatment period of 260 weeks (Up to approximately 272 weeks)

Clinical laboratory tests included the analysis of chemistry, haematology, and urinalysis. Any value outside the normal range will be flagged for the attention of the investigator who will assess whether or not a flagged value is of clinical significance. Only those categories with at least one participant with event are reported. Normal range of creatinine clearance is 110 to 150 mL/min in males and 100 to 130 mL/min in females. Normal urine protein to creatinine (P/C) ratio= \<0.2. Normal range for Alanine Aminotransferase (ALT) is 4 to 36 units per liter (U/L). Platelets normal range=140×10\^9/L to 400×10\^9/L.

Percentage of Participants With Change From Baseline in QT Interval Corrected Using Fridericia's Formula (QTcF) as Determined by Electrocardiogram (ECG)
From first dose of study drug up to 3 months post treatment period of 260 weeks (Up to approximately 272 weeks)

Normal QTcF at Baseline is defined as ≤450 milliseconds (ms) for males or ≤470 ms for females. Percentage of participants with QT interval outside of normal range are reported.

Percentage of Participants Using Concomitant Medication for Nervous and Cardiovascular System Disorders
From first dose of study drug up to 3 months post treatment period of 260 weeks (Up to approximately 272 weeks)

A concomitant therapy was any non-protocol-specified drug or substance (including over-the counter medications, herbal medications, and vitamin supplements) administered between signing of informed consent and the final post-treatment visit for treating nervous and cardiovascular system disorders.

Percentage of Participants With Change From Baseline in Ophthalmic Examination as Assessed by Visual Acuity Changes
From first dose of study drug up to 3 months post treatment period of 260 weeks (Up to approximately 272 weeks)
Percentage of Participants With Change From Baseline in Light Detection Ability Measured by Electroretinography
Baseline (Baseline is the Baseline of the Previous Study- Study CS2), Weeks 78 and 156
Change From Baseline In The Modified Neuropathy Impairment Score (mNIS) +7 Composite Score at Week 66
Baseline and Week 66

The mNIS+7 composite score is a measure of neurologic impairment that evaluates muscle weakness, sensation, reflexes, nerve conduction, and autonomic function. The mNIS+7 Composite Score has a range of -22.32 to 346.32 and a higher mNIS+7 composite score indicates lower function.

Secondary Endpoints

Change From Baseline in Norfolk QOL-DN at Week 35
Baseline, Week 35
Change From Baseline in Neuropathy Symptom and Change (NSC) Score at Weeks 35 and 66
Baseline, Week 35, Week 66
Change From Baseline in the Physical Component Summary (PCS) Score of the 36-Item Short Form Survey (SF-36) at Week 65
Baseline, Week 65
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Study Design & Arms

AllocationRANDOMIZED
MaskingNONE
ModelCROSSOVER
PurposeTREATMENT

Treatment Arms

ArmTypeDescription
InotersenACTIVE_COMPARATORParticipants received inotersen, 300 milligrams (mg), subcutaneously (SC), once weekly up to Week 34. After Week 35 assessment, participants received eplontersen, 45 mg, SC, once every 4 weeks starting from Week 37 up to Week 81.
EplontersenEXPERIMENTALParticipants received eplontersen, 45 mg, SC, once every 4 weeks up to Week 81.
Previous Placebo-Inotersen 300 mgEXPERIMENTALParticipants received subcutaneous (SC) doses of 300 milligrams (mg) inotersen once weekly for up to 260 weeks. Participants who received inotersen-matching placebo in the previous study- ISIS 420915-CS2 (NCT01737398) were included in this group.
Previous Inotersen-Inotersen 300 mgEXPERIMENTALParticipants received SC doses of 300 mg inotersen once weekly for up to 260 weeks. Participants who received inotersen in the previous study- ISIS 420915-CS2 were included in this group.
PlaceboACTIVE_COMPARATORPlacebo administered SC 3 times on alternate days in the first week and then once-weekly for 64 weeks

Interventions

NameTypeDescription
InotersenDRUGInotersen by subcutaneous injection
EplontersenDRUGEplontersen by subcutaneous injection
PlaceboDRUG -
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Eligibility Criteria

Age Range18 Years to 82 Years
SexALL
Healthy VolunteersNo
Study Sites45

Key Inclusion Criteria: 1. Aged 18 to 82 years at the time of informed consent 2. Females must be non-pregnant and non-lactating, and either surgically sterile or post-menopausal or abstinent 3. Males must be surgically sterile or, abstinent or, if engaged in sexual relations with a woman of child-...

Countries:United StatesArgentinaAustraliaBrazilCanadaCyprusFranceGermanyGreeceItalyNew ZealandPortugalSpainSwedenTaiwanTurkey (Türkiye)United Kingdom
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Frequently asked questions about Inotersen

What is Inotersen used for?

Inotersen is used for hereditary transthyretin-mediated amyloid polyneuropathy, also known as familial amyloid polyneuropathy (FAP). It is an investigational small molecule being developed by Ionis Pharmaceuticals, Inc. (IONS) for this rare disease indication.

How does Inotersen work?

Inotersen targets transthyretin (TTR) messenger RNA to reduce production of the transthyretin protein, which forms amyloid deposits in hereditary transthyretin-mediated amyloid polyneuropathy. By lowering TTR levels, it aims to slow or halt the progression of the disease.

Who makes Inotersen?

Inotersen is developed by Ionis Pharmaceuticals, Inc., a biopharmaceutical company traded on NASDAQ under the ticker IONS. The company is conducting clinical trials to evaluate the drug's efficacy and safety in patients with hereditary transthyretin-mediated amyloid polyneuropathy.

What phase is Inotersen in?

Inotersen is in Phase 3 clinical development. Two trials have been completed, including a Phase 2 study and a Phase 3 extension study, with a total enrollment of 308 participants. The drug is investigational and not yet approved by regulatory authorities.

What clinical trials is Inotersen in?

Inotersen has been studied in two completed trials: NCT01737398, a Phase 2 efficacy and safety study in familial amyloid polyneuropathy with 173 participants, and NCT02175004, a Phase 3 extension study assessing long-term safety and efficacy with 135 participants. Both were randomized, double-blind, and active-controlled.

Is Inotersen the same as Eplontersen?

No, Inotersen is not the same as Eplontersen. Eplontersen, formerly known as ION-682884, IONIS-TTR-LRx, and AKCEA-TTR-LRx, is a separate drug evaluated in the NEURO-TTRansform study (NCT04136184) for hereditary transthyretin-mediated amyloid polyneuropathy. Inotersen and Eplontersen are distinct investigational compounds.