NCT03128593

Brief Summary

The purpose of this study in patients with mucopolysaccharidosis type II (MPS II) is below,

  • to collect the safety information of JR-141
  • to evaluate the plasma pharmacokinetics of JR-141
  • to explore the efficacy of JR-141 on MPS II-related central nervous system symptoms and general symptoms

Trial Health

87
On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment
14

participants targeted

Target at below P25 for phase_1

Timeline
Completed

Started Mar 2017

Shorter than P25 for phase_1

Geographic Reach
1 country

8 active sites

Status
completed

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

Study Start

First participant enrolled

March 30, 2017

Completed
11 days until next milestone

First Submitted

Initial submission to the registry

April 10, 2017

Completed
15 days until next milestone

First Posted

Study publicly available on registry

April 25, 2017

Completed
5 months until next milestone

Primary Completion

Last participant's last visit for primary outcome

October 4, 2017

Completed
Same day until next milestone

Study Completion

Last participant's last visit for all outcomes

October 4, 2017

Completed
Last Updated

November 14, 2022

Status Verified

November 1, 2022

Enrollment Period

6 months

First QC Date

April 10, 2017

Last Update Submit

November 10, 2022

Conditions

Outcome Measures

Primary Outcomes (1)

  • Number of participants with Adverse Events

    * Adverse events * Laboratory tests * Vital signs * 12-lead electrocardiogram * Antibody * Infusion associated reaction

    4 weeks

Secondary Outcomes (7)

  • Plasma Pharmacokinetic parameter [Maximum Plasma Concentration [Cmax]]

    4 weeks

  • Plasma Pharmacokinetic parameter [Area Under the Curve [AUC]]

    4 weeks

  • Urinary and serum heparan sulfate (HS) /dermatan sulfate (DS)

    4 weeks

  • HS/DS in CSF

    4 weeks

  • Urinary total GAG

    4 weeks

  • +2 more secondary outcomes

Study Arms (1)

Experimental: JR-141

EXPERIMENTAL
Drug: JR-141

Interventions

JR-141DRUG

IV infusion (lyophilized powder), 0.01-2.0 mg/kg/week

Experimental: JR-141

Eligibility Criteria

Age6 Years+
Sexmale
Healthy VolunteersNo
Age GroupsChild (0-17), Adult (18-64), Older Adult (65+)

You may qualify if:

  • Patients aged 6 years or older at the time of informed consent.
  • Patients diagnosed with MPS II.
  • Patients who have received idursulfase (0.5 mg/kg/week) continuously for at least 12 weeks until the initial dose of JR-141.

You may not qualify if:

  • Patients with a history of hematopoietic stem cell transplantation, excluding those who need enzyme replacement therapy even after hematopoietic stem cell transplantation.
  • Patients in whom lumbar puncture cannot be performed.
  • Patients who have developed serious drug allergy or hypersensitivity that is inappropriate for participation in the study.
  • Patients who have received other investigational products within 4 months before enrollment in the study.

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (8)

Gifu Clinical site

Gifu, 501-1194, Japan

Location

Fukuoka Clinical site

Kurume, 830-0011, Japan

Location

Tokyo clinical site1

Minato, 105-8471, Japan

Location

Osaka Clinical site1

Osaka, 545-8586, Japan

Location

Saitama Clinical site

Saitama, 330-8777, Japan

Location

Tokyo clinical site2

Setagaya City, 157-8535, Japan

Location

Osaka Clinical site2

Suita, 157-8535, Japan

Location

Tottori Clinical site

Yonago, 683-8504, Japan

Location

Related Publications (1)

  • Okuyama T, Eto Y, Sakai N, Minami K, Yamamoto T, Sonoda H, Yamaoka M, Tachibana K, Hirato T, Sato Y. Iduronate-2-Sulfatase with Anti-human Transferrin Receptor Antibody for Neuropathic Mucopolysaccharidosis II: A Phase 1/2 Trial. Mol Ther. 2019 Feb 6;27(2):456-464. doi: 10.1016/j.ymthe.2018.12.005. Epub 2018 Dec 8.

Related Links

MeSH Terms

Conditions

Mucopolysaccharidosis II

Condition Hierarchy (Ancestors)

X-Linked Intellectual DisabilityIntellectual DisabilityNeurobehavioral ManifestationsNeurologic ManifestationsNervous System DiseasesGenetic Diseases, X-LinkedGenetic Diseases, InbornCongenital, Hereditary, and Neonatal Diseases and AbnormalitiesHeredodegenerative Disorders, Nervous SystemMucopolysaccharidosesCarbohydrate Metabolism, Inborn ErrorsMetabolism, Inborn ErrorsLysosomal Storage DiseasesMucinosesConnective Tissue DiseasesSkin and Connective Tissue DiseasesMetabolic DiseasesNutritional and Metabolic Diseases

Study Design

Study Type
interventional
Phase
phase 1
Allocation
NA
Masking
NONE
Purpose
TREATMENT
Intervention Model
SEQUENTIAL
Sponsor Type
INDUSTRY
Responsible Party
SPONSOR

Study Record Dates

First Submitted

April 10, 2017

First Posted

April 25, 2017

Study Start

March 30, 2017

Primary Completion

October 4, 2017

Study Completion

October 4, 2017

Last Updated

November 14, 2022

Record last verified: 2022-11

Locations