Actively Recruiting

Phase 1
Age: 18Years - 80Years
All Genders
ID05241262

Study of N-acetylcysteine for Patients With the m.3243A>G Mutation and Low Brain Glutathione Levels

Led by Michio Hirano, MD · Updated on 2026-01-23

18

Participants Needed

1

Research Sites

N/A

Total Duration

On this page

Sponsors

M

Michio Hirano, MD

Lead Sponsor

U

United States Department of Defense

Collaborating Sponsor

AI-Summary

What this Trial Is About

Researchers are studying the effects of N-Acetylcysteine (NAC), an antioxidant, on patients with the m.3243A>G mitochondrial mutation who often have low brain glutathione levels. This condition may impair the brain's ability to repair damage caused by toxic chemicals related to the mitochondrial disorder. The study aims to assess how NAC affects brain glutathione levels, cognitive skills, motor skills, and overall quality of life. Participants will receive NAC in a dose escalation format, starting with 18 individuals taking either 1800, 3600, or 5400 mg per day, divided into three daily doses, over three months. The study will first determine if the 3600 mg dose is safe and shows potential effectiveness. If it is safe, further participants will receive 5400 mg per day; if not, the lower 1800 mg dose will be used. This pilot study helps identify the safest and most effective NAC dose for future trials. During the study, participants will be monitored over four months to assess the maximum tolerated dose of NAC, changes in neurological and cognitive function, motor capacity using a 6-minute walk test, performance scale scores, and brain glutathione levels. Researchers will use magnetic resonance spectroscopic imaging (MRSI) and various clinical assessments to evaluate these outcomes, ensuring close observation of safety and treatment effects throughout the trial.

CONDITIONS

Brief Title

Study of N-acetylcysteine in the Treatment of Patients With the m.3243A>G Mutation and Low Brain Glutathione Levels

Who Can Participate

Age: 18Years - 80Years
All Genders

Eligibility Criteria

Eligible

You may qualify if you...

  • Ages 18 to 80 years
  • Low brain glutathione levels confirmed by magnetic resonance spectroscopic imaging (MRSI)
  • Carriers or suspected carriers of the m.3243A>G mitochondrial mutation with genetic confirmation before starting NAC
Not Eligible

You will not qualify if you...

  • Normal brain glutathione levels
  • Pregnant or lactating individuals
  • Medically unstable as determined by the Principal Investigator
  • Allergy to NAC or other sulfur-containing drugs
  • Inability to follow the study protocol

AI-Screening

AI-Powered Screening

Complete this quick 3-step screening to check your eligibility

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Your Study Journey

Screening

Duration - 2 to 4 weeks

Participants are screened for eligibility to participate in the trial.

Treatment

Duration - 3 months

Participants receive N-Acetylcysteine (NAC) at doses of 1800, 3600 or 5400 mg per day divided into 3 daily doses to improve brain glutathione levels and cognitive and motor skills.

Trial Site Locations

Total: 1 location

1

Columbia University Irving Medical Center

New York, New York, United States, 10032

Actively Recruiting

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Research Team

K

Kris Engelstad, MS, CGC

How is the study designed?

Study Type

INTERVENTIONAL

Masking

NONE

Allocation

NA

Model

SINGLE_GROUP

Primary Purpose

TREATMENT

Number of Arms

1

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