Diet Treatment Glucose Transporter Type 1 Deficiency (G1D)

This is an open-label, single arm trial of orally-administered C7 in G1D. Subjects will
replace a fixed percentage of their daily caloric intake (based on the results of Protocol 1)
with C7 for 6 months, undergo full evaluation and discontinuation of treatment at a 6 month
visit, and return for an off-treatment follow up visit 3 months after C7 oil discontinuation,
for total duration of participation of 9 months. Subjects will undergo treatment initiation
on a 24-hour inpatient basis. During that 24-hr inpatient treatment initiation, subjects will
have continuous EEG both to monitor for real-time seizure activity (for safety) and to
determine EEG changes (secondary outcome) before, during, and after treatment initiation.
Subjects will undergo clinical evaluation, comprehensive blood work, ataxia scale rating,
EEG, and neuropsychological testing at baseline, 6 months, and 9 months.

Inclusion Criteria:

- Diagnosis of glucose transporter type I deficiency (G1D), confirmed by clinical
genotyping at a CLIA-certified laboratory or by PET scan.

- Stable diet on no dietary therapy (i.e., no dietary therapy for 1 month).

- Males and females 30 months to 17 years 11 months old, inclusive.

Exclusion Criteria:

- Subjects with evidence of independent, unrelated metabolic and/or genetic disease.

- Subjects with a chronic gastrointestinal disorder, such as irritable bowel syndrome,
Crohn's disease, or colitis that could increase the subject's risk of developing
diarrhea or stomach pain.

- Subjects with a BMI (body mass index) greater than or equal to 30.

- Subjects currently on dietary therapy (i.e., ketogenic diet, medium chain triglyceride
supplemented diets, Atkins diet and versions of it, low glycemic index diet, and
related diets).

- Subjects with no evidence of abnormal EEG (spike wave discharges) in the last 12
months.

- Women who are pregnant or breast-feeding may not participate. Women who plan to become
pregnant during the course of the study, or who are unwilling to use birth control to
prevent pregnancy (including abstinence) may not participate. Females age 10 and over
will be asked to provide a urine sample for a pregnancy test via dipstick. Subjects
will be asked to agree to abstinence or another form of birth control for the duration
of the study.

- Allergy/sensitivity to C7.

- Previous use of triheptanoin in the past 1 month. Subjects who participate in Protocol
1 of this study are thus eligible.

- Subjects exhibiting signs of dementia, or diagnosed with any degenerative brain
disorder (such as Alzheimer's disease) that would confound assessment of cognitive
changes, in the opinion of the investigator.

- Active drug or alcohol use or dependence that, in the opinion of the investigator,
would interfere with adherence to study requirements.

- Inability or unwillingness of subject or legal guardian/representative to give written
informed consent, or assent for children age 10-17.

- Addition of a new antiseizure drug in the previous 3 months.