glycerol has been researched along with Zellweger Syndrome in 2 studies
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Zellweger Syndrome: An autosomal recessive disorder due to defects in PEROXISOME biogenesis which involves more than 13 genes encoding peroxin proteins of the peroxisomal membrane and matrix. Zellweger syndrome is typically seen in the neonatal period with features such as dysmorphic skull; MUSCLE HYPOTONIA; SENSORINEURAL HEARING LOSS; visual compromise; SEIZURES; progressive degeneration of the KIDNEYS and the LIVER. Zellweger-like syndrome refers to phenotypes resembling the neonatal Zellweger syndrome but seen in children or adults with apparently intact peroxisome biogenesis.
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 1 (50.00) | 18.2507 |
| 2000's | 0 (0.00) | 29.6817 |
| 2010's | 1 (50.00) | 24.3611 |
| 2020's | 0 (0.00) | 2.80 |
| Authors | Studies |
|---|---|
| Zhang, R | 1 |
| Chen, L | 1 |
| Jiralerspong, S | 1 |
| Snowden, A | 1 |
| Steinberg, S | 1 |
| Braverman, N | 1 |
| Hoefler, G | 1 |
| Paschke, E | 1 |
| Hoefler, S | 1 |
| Moser, AB | 1 |
| Moser, HW | 1 |
| Trial | Phase | Enrollment | Study Type | Start Date | Status | ||
|---|---|---|---|---|---|---|---|
| A Pilot, Open Label Trial Assessing the Safety and Efficacy of Betaine in Children With Peroxisome Biogenesis Disorders.[NCT01838941] | Phase 3 | 12 participants (Actual) | Interventional | 2013-03-31 | Completed | ||
| [information is prepared from clinicaltrials.gov, extracted Sep-2024] | |||||||
C26/C22 ratio in plasma is a recognized biomarker for very long chain fatty acid (normal range: 0.002-0.018). It was measured twice before the beginning of treatment and measured once at the end. (NCT01838941)
Timeframe: 6 months
| Intervention | ratio (Mean) | |
|---|---|---|
| Baseline | At 6 months | |
| Betaine | 0.180 | 0.188 |
2 other studies available for glycerol and Zellweger Syndrome
| Article | Year |
|---|---|
Recovery of PEX1-Gly843Asp peroxisome dysfunction by small-molecule compounds.
Topics: Adenosine Triphosphatases; Amino Acid Substitution; ATPases Associated with Diverse Cellular Activit | 2010 |
Photosensitized killing of cultured fibroblasts from patients with peroxisomal disorders due to pyrene fatty acid-mediated ultraviolet damage.
Topics: Adrenoleukodystrophy; Cell Survival; Cells, Cultured; Fatty Acids; Fibroblasts; Glycerol; Humans; Mi | 1991 |