NCT03122925

TCA Cycle in the Dentate in Friedreich's Ataxia

Measurement of the TCA Cycle Rate in the Dentate Nucleus in Friedreich's Ataxia

• Status: Completed
• Phase: —
• Study type: Observational
• Enrollment: 20 (actual)
• Sponsor: University of Minnesota · Academic / Other
• Sex: All
• Age: 18 Years
• Healthy volunteers: Accepted

Summary

OBJECTIVE: To measure the tricarboxylic acid (TCA) cycle rate in the dentate nucleus in a group of control subjects and subjects with Friedreich's Ataxia (FRDA). HYPOTHESIS: The TCA cycle rate will be lower in FRDA subjects than in controls APPROACH: The investigators will infuse carbon-13 (13C) labeled glucose and measure the rate of 13C label incorporation from glucose to glutamate in the brain using in vivo magnetic resonance spectroscopy.

Detailed description

The investigators will measure the TCA cycle rate in the dentate nucleus in a group of FRDA patients and in a group of age-matched healthy controls using 13C MRS in vivo together with systemic i.v. infusion of 13C-labeled glucose. The investigators aim to obtain adequate data in 16 subjects grouped as follows: * n=4 pilot subjects (healthy subjects) for testing and optimization of the experimental setup. * n=6 healthy controls * n=6 FRDA patients In other to reach this goal, the investigators plan to recruit up to 20 subjects, in case some subjects withdraw before completion of the MR scan, or in case the measured data are not adequate (e.g. technical problem with the scanner).

Conditions

• Friedreich Ataxia

Interventions

Type	Name	Description
DEVICE	MR	MR

Timeline

• Start date: 2017-08-29
• Primary completion: 2023-01-11
• Completion: 2023-01-11
• First posted: 2017-04-21
• Last updated: 2023-03-27

Locations

1 site across 1 country: United States

Regulatory

• FDA-regulated device study