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Rhttp1 (CERLIPONASE ALFA)
Rhttp1 (generic name: CERLIPONASE ALFA) is a Hydrolytic Lysosomal N-terminal Tripeptidyl Peptidase Enzyme drug developed by BioMarin. It is currently FDA-approved (first approved 2017) for Late-infantile neuronal ceroid lipofuscinosis.
Cerliponase alfa works by inhibiting the breakdown of a protein called tripeptidyl peptidase, which is deficient in patients with late-infantile neuronal ceroid lipofuscinosis.
Cerliponase alfa, also known as Relyvio, is a small molecule drug developed by Biogen (previously by Biogen and now by Biogen's competitor) and currently owned by Biogen. It is a hydrolytic lysosomal N-terminal tripeptidyl peptidase inhibitor, approved by the FDA in 2017 for the treatment of late-infantile neuronal ceroid lipofuscinosis. This rare genetic disorder affects the brain and causes progressive vision loss, seizures, and loss of motor skills. Cerliponase alfa is administered intravenously and is not yet available as a generic version. Key safety considerations include the risk of infusion-related reactions and the potential for immune system activation.
At a glance
| Generic name | CERLIPONASE ALFA |
|---|---|
| Sponsor | BioMarin |
| Drug class | Hydrolytic Lysosomal N-terminal Tripeptidyl Peptidase |
| Modality | Enzyme |
| Therapeutic area | Neuroscience |
| Phase | FDA-approved |
| First approval | 2017 |
Mechanism of action
Imagine your brain has a recycling system that breaks down old or damaged proteins. In people with this disorder, this system doesn't work properly, leading to a buildup of toxic waste. Cerliponase alfa helps by blocking the enzyme that breaks down a specific protein, allowing the brain to recycle it more efficiently.
Approved indications
- Late-infantile neuronal ceroid lipofuscinosis
Boxed warnings
- WARNING: HYPERSENSITIVITY REACTIONS INCLUDING ANAPHYLAXIS Patients treated with enzyme replacement therapies have experienced life-threatening hypersensitivity reactions, including anaphylaxis. Anaphylaxis has occurred during the early course of enzyme replacement therapy and after extended duration of therapy. Initiate BRINEURA in a healthcare setting with appropriate medical monitoring and support measures, including access to cardiopulmonary resuscitation equipment. If a severe hypersensitivity reaction (e.g., anaphylaxis) occurs, discontinue BRINEURA and immediately initiate appropriate medical treatment, including use of epinephrine. Inform patients of the symptoms of life-threatening hypersensitivity reactions, including anaphylaxis and to seek immediate medical care should symptoms occur [see Warnings and Precautions (5.1) ]. WARNING: HYPERSENSITIVITY REACTIONS INCLUDING ANAPHYLAXIS See full prescribing information for complete boxed warning Anaphylaxis has occurred during the early course of enzyme replacement therapy and after extended duration of therapy. ( 5.1 ) Initiate BRINEURA in a healthcare setting with appropriate medical monitoring and support measures, including access to cardiopulmonary resuscitation equipment. ( 5.1 ) If a severe hypersensitivity reaction (e.g., anaphylaxis) occurs, discontinue BRINEURA and immediately initiate appropriate medical treatment, including use of epinephrine. ( 5.1 )
Common side effects
- Seizures
- Device-related complications
- Hypersensitivity
- Pyrexia
- Hematoma
- Pleocytosis
- Vomiting
- ECG abnormalities
- Decreased CSF protein
- Device-related infections
- Increased CSF protein
- Headache
Serious adverse events
- Intraventricular access device-related CNS infections
- Hypoxia
- Device leakage
- Low mean arterial pressure
- Decreased oxygen saturation
Key clinical trials
- Cerliponase Alfa Observational Study in the US
- Examining Developmental Outcomes of Children Diagnosed With CLN2 Disease
- Intravitreal ERT to Prevent Retinal Disease Progression in Children With CLN2 (PHASE1,PHASE2)
- A Safety, Tolerability, and Efficacy Study of BMN 190 in Pediatric Patients < 18 Years of Age With CLN2 Disease (PHASE2)
- An Extension Study to Evaluate the Long-Term Efficacy and Safety of BMN 190 in Patients With CLN2 Disease (PHASE1,PHASE2)
- Natural History and Longitudinal Clinical Assessments in NCL / Batten Disease, the International DEM-CHILD Database
- A Phase 1/2 Open-Label Dose-Escalation Study to Evaluate Safety, Tolerability, Pharmacokinetics, and Efficacy of Intracerebroventricular BMN 190 in Patients With Late-Infantile Neuronal Ceroid Lipofuscinosis (CLN2) Disease (PHASE1,PHASE2)
Primary sources
Every claim on this page is sourced from regulatory or scientific primary sources. See our editorial policy for full methodology.
| Source | Used for |
|---|---|
| FDA label | Mechanism, indications, dosing, boxed warnings, drug interactions |
| ClinicalTrials.gov | Trial enrolment, design, endpoints, results |
Competitive intelligence
For the full competitive landscape — auto-detected comparators, recent regulatory actions across the set, upcoming PDUFA, patent timeline, sponsor landscape:
- Rhttp1 CI brief — competitive landscape report
- Rhttp1 updates RSS · CI watch RSS
- BioMarin portfolio CI
Frequently asked questions about Rhttp1
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Related
- Drug class: All Hydrolytic Lysosomal N-terminal Tripeptidyl Peptidase drugs
- Manufacturer: BioMarin — full pipeline
- Therapeutic area: All drugs in Neuroscience
- Indication: Drugs for Late-infantile neuronal ceroid lipofuscinosis
- Compare: Rhttp1 vs similar drugs
- Pricing: Rhttp1 cost, discount & access