Angelman Syndrome (AS) Biomarker Study

NCT ID: NCT04103333

Last Updated: 2022-01-28

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

COMPLETED

Clinical Phase

EARLY_PHASE1

Total Enrollment

20 participants

Study Classification

INTERVENTIONAL

Study Start Date

2019-12-18

Study Completion Date

2022-01-02

Brief Summary

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The primary objective of this study is to measure ubiquitin-protein ligase E3A (UBE3A) protein levels in cerebrospinal fluid (CSF) and to evaluate its utility as a biomarker in support of the development of therapies for AS.

Detailed Description

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Conditions

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Angelman Syndrome Chromosome 15q Duplication (dup15q) Syndrome

Study Design

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Allocation Method

NON_RANDOMIZED

Intervention Model

PARALLEL

Primary Study Purpose

DIAGNOSTIC

Blinding Strategy

NONE

Study Groups

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Angelman Syndrome: Group 1

Participants aged 0-6 years old with Angelman Syndrome will undergo lumbar puncture (LP) for CSF and blood collection while under general anesthesia or conscious sedation during or after a prescheduled procedure unrelated to the study.

Group Type EXPERIMENTAL

Lumbar Puncture

Intervention Type PROCEDURE

Administered as specified in the treatment arm.

Blood Collection

Intervention Type PROCEDURE

Administered as specified in the treatment arm.

Angelman Syndrome: Group 2

Participants aged 7-12 years old with Angelman Syndrome will undergo lumbar puncture (LP) for CSF and blood collection while under general anesthesia or conscious sedation during or after a prescheduled procedure unrelated to the study.

Group Type EXPERIMENTAL

Lumbar Puncture

Intervention Type PROCEDURE

Administered as specified in the treatment arm.

Blood Collection

Intervention Type PROCEDURE

Administered as specified in the treatment arm.

Angelman Syndrome: Group 3

Participants aged 13-18 years old with Angelman Syndrome will undergo lumbar puncture (LP) for CSF and blood collection while under general anesthesia or conscious sedation during or after a prescheduled procedure unrelated to the study.

Group Type EXPERIMENTAL

Lumbar Puncture

Intervention Type PROCEDURE

Administered as specified in the treatment arm.

Blood Collection

Intervention Type PROCEDURE

Administered as specified in the treatment arm.

Angelman Syndrome: Group 4

Participants aged 19-50 years old with Angelman Syndrome will undergo lumbar puncture (LP) for CSF and blood collection while under general anesthesia or conscious sedation during or after a prescheduled procedure unrelated to the study.

Group Type EXPERIMENTAL

Lumbar Puncture

Intervention Type PROCEDURE

Administered as specified in the treatment arm.

Blood Collection

Intervention Type PROCEDURE

Administered as specified in the treatment arm.

Dup15q Syndrome: Group 1

Participants aged 0-6 years old with Dup15q Syndrome will undergo lumbar puncture (LP) for CSF and blood collection while under general anesthesia or conscious sedation during or after a prescheduled procedure unrelated to the study.

Group Type EXPERIMENTAL

Lumbar Puncture

Intervention Type PROCEDURE

Administered as specified in the treatment arm.

Blood Collection

Intervention Type PROCEDURE

Administered as specified in the treatment arm.

Dup15q Syndrome: Group 2

Participants aged 7-12 years old with Dup15q Syndrome will undergo lumbar puncture (LP) for CSF and blood collection while under general anesthesia or conscious sedation during or after a prescheduled procedure unrelated to the study.

Group Type EXPERIMENTAL

Lumbar Puncture

Intervention Type PROCEDURE

Administered as specified in the treatment arm.

Blood Collection

Intervention Type PROCEDURE

Administered as specified in the treatment arm.

Dup15q Syndrome: Group 3

Participants aged 13-18 years old with Dup15q Syndrome will undergo lumbar puncture (LP) for CSF and blood collection while under general anesthesia or conscious sedation during or after a prescheduled procedure unrelated to the study.

Group Type EXPERIMENTAL

Lumbar Puncture

Intervention Type PROCEDURE

Administered as specified in the treatment arm.

Blood Collection

Intervention Type PROCEDURE

Administered as specified in the treatment arm.

Dup15q Syndrome: Group 4

Participants aged 19-50 years old with Dup15q Syndrome will undergo lumbar puncture (LP) for CSF and blood collection while under general anesthesia or conscious sedation during or after a prescheduled procedure unrelated to the study.

Group Type EXPERIMENTAL

Lumbar Puncture

Intervention Type PROCEDURE

Administered as specified in the treatment arm.

Blood Collection

Intervention Type PROCEDURE

Administered as specified in the treatment arm.

Interventions

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Lumbar Puncture

Administered as specified in the treatment arm.

Intervention Type PROCEDURE

Blood Collection

Administered as specified in the treatment arm.

Intervention Type PROCEDURE

Eligibility Criteria

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Inclusion Criteria

* Ability of the participant's legally authorized representative (LAR) to understand the purpose and risks of the study and provide signed and dated informed consent and authorization to use confidential health information in accordance with national and local privacy regulations
* Must have genetically confirmed diagnosis of AS (UBE3A deletion, UBE3A mutation, paternal uniparental disomy, or imprinting center defect) or dup15q syndrome (with number and size of duplications of 15q specified) provided by the Investigator
* Must be scheduled for a procedure unrelated to the study that will involve administration of general anesthesia or conscious sedation.

Exclusion Criteria

* Lumbar Puncture (LP) procedure less than 30 days before the Sampling Visit
* Any contraindications to having an LP
* The blood and CSF collection will, in the opinion of the Investigator, inhibit, in some way, the prescheduled procedure that requires anesthesia or sedation
* Current enrollment or past enrollment in an interventional clinical study in which an investigational gene therapy or antisense oligonucleotide (ASO) is/was administered
* Enrollment in an interventional clinical study in which an investigational small molecule/antibody treatment or approved small molecule/antibody therapy is administered within 1 month (or 5 half-lives of study agent, whichever is longer) prior to the Sampling Visit which, under the judgement of the Investigator and/or Sponsor would affect UBE3A and other CSF biomarker levels. Use of approved or investigational small molecule therapies which would not impact the biomarkers above will be eligible with Sponsor approval prior to enrollment (e.g., anti-epileptic drugs being studied in alternative formulation, other derivatives of benzodiazepines, or other same class drugs as those already permitted as part of the study).
Maximum Eligible Age

50 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Biogen

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Medical Director

Role: STUDY_DIRECTOR

Biogen

Locations

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Rady Childrens Hospital

San Diego, California, United States

Site Status

Rush Medical College

Chicago, Illinois, United States

Site Status

Boston Children's Hospital

Boston, Massachusetts, United States

Site Status

University of North Carolina Hospital

Carolina, North Carolina, United States

Site Status

Vanderbilt University Medical Center

Nashville, Tennessee, United States

Site Status

Countries

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United States

Other Identifiers

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992AN001

Identifier Type: -

Identifier Source: org_study_id

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