Genetics of Charcot Marie Tooth (CMT) - Modifiers of CMT1A, New Causes of CMT2

NCT ID: NCT01193088

Last Updated: 2025-10-07

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

Get a concise snapshot of the trial, including recruitment status, study phase, enrollment targets, and key timeline milestones.

Recruitment Status

RECRUITING

Total Enrollment

1050 participants

Study Classification

OBSERVATIONAL

Study Start Date

2010-05-31

Study Completion Date

2026-12-31

Brief Summary

Review the sponsor-provided synopsis that highlights what the study is about and why it is being conducted.

This project includes two projects. One is looking for new genes that cause Charcot Marie Tooth disease (CMT). The other is looking for genes that do not cause CMT, but may modify the symptoms a person has.

Detailed Description

Dive into the extended narrative that explains the scientific background, objectives, and procedures in greater depth.

This project is to understand modifier genes and how they influence the severity of disease expression, along with identifying new forms of CMT which have not been genetically determined. Subjects who are eligible will either have CMT type 1A (CMT1A) or an unknown form of CMT. Blood will be drawn and sent to the University of Miami where they receive the coded sample and process it through exome sequencing. Subjects will be told that this is optional.

Conditions

See the medical conditions and disease areas that this research is targeting or investigating.

Charcot-Marie-Tooth Disease, Type Ia (Disorder) HMSN

Study Design

Understand how the trial is structured, including allocation methods, masking strategies, primary purpose, and other design elements.

Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Study Groups

Review each arm or cohort in the study, along with the interventions and objectives associated with them.

CMT1A

Families/people with genetically defined CMT1A

No interventions assigned to this group

Genetically undefined CMT

Families/people with genetically undefined CMT with common causes ruled out.

No interventions assigned to this group

Eligibility Criteria

Check the participation requirements, including inclusion and exclusion rules, age limits, and whether healthy volunteers are accepted.

Inclusion Criteria

All patients must agree to take part in the study and sign a consent form. A teenager (age 13-17 years) considering enrolling must agree to take part in the study and sign an assent form (depending on local ethics committee requirements).



Patients must have at least one of the following:

1. Patient has a documented PMP22 duplication. AND/OR
2. Patient has a first or second degree relative (parent, child, sibling, half- sibling, aunt, uncle, grandparent, grandchild, niece, or nephew) with a documented PMP22 duplication AND a clear link between that family member and the affected patient AND a phenotype consistent with CMT1A.

i. A clear link is necessary for a second-degree relative. For example, if a grandparent is affected and has a PMP22 duplication, and the parent does not have any signs, symptoms, or electrophysiology consistent with CMT1A, there is no clear link.

ii. In cases where clear links are not available, genetic testing is required for the patient or the first degree family member who is not clearly affected.


a. Patient has demonstrated neuropathy on nerve conduction studies or clinically diagnosed genetic neuropathy, in the opinion of the investigator or genetic counsellor.


1. Person is a family member of a CMT patient who is enrolled in the CMT Exome Project.

AND one of the following:
2. Person does not have a peripheral neuropathy, in the opinion of the investigator or genetic counsellor.

OR
3. Person is suspected to have a peripheral neuropathy, but has not been examined at an INC site.

Exclusion Criteria

1. Patient does not wish to participate or does not sign a consent form.
2. For CMT Exome Project, patient has a genetically confirmed form of CMT (i.e. mutation in MFN2 causing CMT2A, mutation in GARS causing CMT2D, etc.).
3. Patients with known neuropathy from a non-genetic source, such as chemotherapies (i.e. Vincristine, Taxol, Cisplatin), diabetes, alcoholism will be evaluated independently so that genetic contributions to their effects on CMT1A phenotypes can also be analyzed.
Eligible Sex

ALL

Accepts Healthy Volunteers

Yes

Sponsors

Meet the organizations funding or collaborating on the study and learn about their roles.

National Institute of Neurological Disorders and Stroke (NINDS)

NIH

Sponsor Role collaborator

Muscular Dystrophy Association

OTHER

Sponsor Role collaborator

University of Rochester

OTHER

Sponsor Role collaborator

University of Pennsylvania

OTHER

Sponsor Role collaborator

King's College Hospital NHS Trust

OTHER

Sponsor Role collaborator

Sydney Children's Hospitals Network

OTHER

Sponsor Role collaborator

Children's Hospital of Philadelphia

OTHER

Sponsor Role collaborator

University of Miami

OTHER

Sponsor Role collaborator

Johns Hopkins University

OTHER

Sponsor Role collaborator

Fondazione I.R.C.C.S. Istituto Neurologico Carlo Besta

OTHER

Sponsor Role collaborator

Cedars-Sinai Medical Center

OTHER

Sponsor Role collaborator

Nemours Children's Clinic

OTHER

Sponsor Role collaborator

Stanford University

OTHER

Sponsor Role collaborator

University of Minnesota

OTHER

Sponsor Role collaborator

Massachusetts General Hospital

OTHER

Sponsor Role collaborator

University of Colorado, Denver

OTHER

Sponsor Role collaborator

Children's National Research Institute

OTHER

Sponsor Role collaborator

University of Michigan

OTHER

Sponsor Role collaborator

St. Jude Children's Research Hospital

OTHER

Sponsor Role collaborator

Connecticut Children's Medical Center

OTHER

Sponsor Role collaborator

Seattle Children's Hospital

OTHER

Sponsor Role collaborator

The Hospital for Sick Children

OTHER

Sponsor Role collaborator

University of Iowa

OTHER

Sponsor Role lead

Responsible Party

Identify the individual or organization who holds primary responsibility for the study information submitted to regulators.

Michael Shy

Professor

Responsibility Role PRINCIPAL_INVESTIGATOR

Principal Investigators

Learn about the lead researchers overseeing the trial and their institutional affiliations.

Michael E Shy, MD

Role: PRINCIPAL_INVESTIGATOR

University of Iowa

Locations

Explore where the study is taking place and check the recruitment status at each participating site.

Cedars-Sinai Medical Center

Los Angeles, California, United States

Site Status RECRUITING

Stanford University

Palo Alto, California, United States

Site Status RECRUITING

University of Colorado Hospital

Aurora, Colorado, United States

Site Status RECRUITING

Connecticut Children's Medical Center

Hartford, Connecticut, United States

Site Status RECRUITING

University of Miami

Miami, Florida, United States

Site Status RECRUITING

University of Iowa

Iowa City, Iowa, United States

Site Status RECRUITING

Johns Hopkins University

Baltimore, Maryland, United States

Site Status RECRUITING

Harvard/Massachusetts General Hospital

Boston, Massachusetts, United States

Site Status RECRUITING

University of Michigan

Ann Arbor, Michigan, United States

Site Status RECRUITING

University of Minnesota

Maple Grove, Minnesota, United States

Site Status RECRUITING

University of Rochester

Rochester, New York, United States

Site Status RECRUITING

University of North Carolina

Chapel Hill, North Carolina, United States

Site Status RECRUITING

Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, United States

Site Status RECRUITING

University of Pennsylvania

Philadelphia, Pennsylvania, United States

Site Status RECRUITING

St. Jude Children's Research Hospital

Memphis, Tennessee, United States

Site Status RECRUITING

Houston Methodist Hospital

Houston, Texas, United States

Site Status RECRUITING

Seattle Children's Hospital

Seattle, Washington, United States

Site Status RECRUITING

Children's Hospital of Westmead

Sydney, New South Wales, Australia

Site Status RECRUITING

The Hospital for Sick Children

Toronto, Ontario, Canada

Site Status RECRUITING

C. Besta Neurological Institute

Milan, , Italy

Site Status RECRUITING

National Hospital of Neurology and Neurosurgery

London, England, United Kingdom

Site Status RECRUITING

Dubowitz Neuromuscular Centre

London, , United Kingdom

Site Status RECRUITING

Countries

Review the countries where the study has at least one active or historical site.

United States Australia Canada Italy United Kingdom

Central Contacts

Reach out to these primary contacts for questions about participation or study logistics.

Tiffany Grider, MS, CGC

Role: CONTACT

319-384-6362

Nicole Kressin, MS, CGC

Role: CONTACT

319-384-6362

Facility Contacts

Find local site contact details for specific facilities participating in the trial.

Marcela Arenas Sanchez

Role: primary

800-233-2771

Veronica Stevens

Role: primary

650-721-5588

Study Recruiter

Role: primary

303-724-4644

Jennifer Twatchtman-Bassett

Role: primary

860-837-7500

Recruiter

Role: primary

305-243-2550

Tiffany Grider, MS, CGC

Role: primary

319-384-6362

Simone Thomas

Role: primary

410-614-4188

Natalie Grant

Role: primary

617-643-6996

Angela Stovall

Role: primary

734-647-4787

Sarah Hilbert, MS

Role: primary

612-624-5978

Janet Sowden

Role: primary

585-275-1267

Said Alhassan

Role: primary

919-966-8175

Sabrina Yum, MD

Role: primary

215-590-1719

Pooja Patel

Role: primary

Colin Quillivan

Role: primary

901-595-7708

Samia Saad

Role: primary

346.238.2287

Shawna Feely

Role: primary

206-987-6678

Gabrielle Donlevy

Role: primary

+61 2 9845 1904

Magdalena Lovaglio

Role: primary

xxx-xxx-xxxx

Daniella Calabrese

Role: primary

+39-02 2394 3001

Matilde Laura, MD

Role: primary

044 207 837 3611

Hinal Patel

Role: primary

+44 0 20 7905 2608

References

Explore related publications, articles, or registry entries linked to this study.

Montenegro G, Powell E, Huang J, Speziani F, Edwards YJ, Beecham G, Hulme W, Siskind C, Vance J, Shy M, Zuchner S. Exome sequencing allows for rapid gene identification in a Charcot-Marie-Tooth family. Ann Neurol. 2011 Mar;69(3):464-70. doi: 10.1002/ana.22235. Epub 2011 Jan 20.

Reference Type RESULT
PMID: 21254193 (View on PubMed)

Related Links

Access external resources that provide additional context or updates about the study.

https://www.rarediseasesnetwork.org/cms/inc

Inherited Neuropathies Consortium Website

Other Identifiers

Review additional registry numbers or institutional identifiers associated with this trial.

1U54NS065712-01

Identifier Type: NIH

Identifier Source: secondary_id

View Link

INC-6602

Identifier Type: -

Identifier Source: org_study_id

More Related Trials

Additional clinical trials that may be relevant based on similarity analysis.

Genetic Study of Sitosterolemia
NCT00004481 COMPLETED