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N/A N=65 Randomized Quadruple-blind Other

CoA-Z in Pantothenate Kinase-associated Neurodegeneration (PKAN)

Pantothenate Kinase-Associated Neurodegeneration

Enrolled (actual)
65
Serious AEs
35.5%
Results posted
Oct 2025
Primary outcome: Primary: Number of Treatment-emergent Adverse Events Assessed Using CTCAE v4.0 — 1.15; 1.38; 1.14; 0.86 Events per person — p=0.50

Study Design & Population

Study type
Interventional
Phase
N/A
Interventions
CoA-Z (Other); Placebo (Other)
Age
Pediatric, Adult, Older Adult · 0+ yrs
Sex
All
Sponsor
Oregon Health and Science University
Primary completion
Sep 2024

Outcome Measures

OutcomeResultp-value
PRIMARY
Number of Treatment-emergent Adverse Events Assessed Using CTCAE v4.0
1.15; 1.38; 1.14; 0.86 0.50
PRIMARY
Number of Treatment-emergent Clinically Significant Laboratory Abnormalities on Complete Blood Count.
0; 1; 0; 0
PRIMARY
Number of Treatment-emergent Clinically Significant Laboratory Abnormalities on Comprehensive Metabolic Profile.
1; 1; 0; 0
PRIMARY
Number of Participants Retained in Each Arm.
15; 16; 17; 15
PRIMARY
Mean Percent of Study Product Consumed.
98.98; 99.25; 98.66; 96.94
SECONDARY
CoASY mRNA Expression
1.31; 1.13; 1.04; 1.07; 1.07; 0.97 0.105

Summary

The purpose of this study is to learn more about how people with the condition pantothenate kinase-associated neurodegeneration (PKAN) respond to a specialized study product. We are hoping to find out if the study product is safe, what effects-good and bad-the study product causes, and whether the study product changes certain measures of disease in PKAN.

Eligibility Criteria

Inclusion Criteria

  • Has a diagnosis of PKAN confirmed by: a) genetic testing confirming 2 pathogenic or likely pathogenic mutations, or (b) typical findings on exam and brain MR imaging with only one pathogenic mutation +/- a second likely pathogenic or VOUS in PANK2, or (c) typical findings on exam and brain MR imaging with a single likely pathogenic or VOUS in PANK2, or (d) be a symptomatic sibling of a proband subject meeting a, b or c.
  • Be between 3 months old and 89 years old.
  • Be able to take study product by mouth or feeding tube.
  • Be willing and able to complete study procedures / telephone visits / blood draws independently, OR have a caregiver / parent willing and able to assist with these tasks.
  • Be enrolled or willing to enroll in the PKANready natural history study (eIRB 10832).
  • Be resident in North America (US or Canada) for the duration of the trial.

Exclusion Criteria

  • Have had exposure to a putative PANK2 bypass therapeutic agent in the 30 days prior to screening.
  • Be concurrently enrolled in another interventional clinical trial.
  • Have concurrent medical or other condition expected to preclude completion of study procedures of confound the assessment of clinical and laboratory measures of safety.
View full record on ClinicalTrials.gov →

Data sourced from ClinicalTrials.gov (NCT04182763). Outcome figures and adverse-event rates are extracted automatically from the registry's posted results and are provided for clinician reference, not as a substitute for the primary publication.

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