| Title |
“Lorenzo’s Oil” Therapy for X-linked Adrenoleukodystrophy: Rationale and Current Assessment of Efficacy
|
|---|---|
| Published in |
Journal of Molecular Neuroscience, August 2007
|
| DOI | 10.1007/s12031-007-0041-4 |
| Pubmed ID | |
| Authors |
Hugo W. Moser, Ann B. Moser, Kim Hollandsworth, N. Hong Brereton, Gerald V. Raymond |
| Abstract |
X-linked adrenoleukodystrophy (X-ALD) is a genetic disorder that damages the nervous system and is associated with the accumulation of saturated very long chain fatty acids (SVLCFA). Oral administration of "Lorenzo's oil" (LO), a 4:1 mixture of glyceryl trioleate and glyceryl trierucate, normalizes the SVLCFA levels in plasma, but its clinical efficacy and the clinical indications for its use have been controversial for more than 15 years. We review the biochemical effects of LO administration and the rationale for its use and present a current appraisal of its capacity to reduce the risk for the childhood cerebral phenotype when administered to asymptomatic boys and to slow progression of adrenomyeloneuropathy in patients without cerebral involvement. We also present current efforts to provide definitive evaluation of its clinical efficacy and discuss its possible role in the new therapeutic opportunities that will arise if newborn screening for X-ALD is validated and implemented. |
Mendeley readers
The data shown below were compiled from readership statistics for 70 Mendeley readers of this research output. Click here to see the associated Mendeley record.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| United States | 2 | 3% |
| Unknown | 68 | 97% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Student > Bachelor | 17 | 24% |
| Student > Master | 9 | 13% |
| Researcher | 7 | 10% |
| Student > Ph. D. Student | 6 | 9% |
| Other | 5 | 7% |
| Other | 10 | 14% |
| Unknown | 16 | 23% |
| Readers by discipline | Count | As % |
|---|---|---|
| Medicine and Dentistry | 14 | 20% |
| Agricultural and Biological Sciences | 10 | 14% |
| Biochemistry, Genetics and Molecular Biology | 9 | 13% |
| Neuroscience | 9 | 13% |
| Pharmacology, Toxicology and Pharmaceutical Science | 3 | 4% |
| Other | 8 | 11% |
| Unknown | 17 | 24% |
Attention Score in Context
This research output has an Altmetric Attention Score of 3. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 24 March 2020.
All research outputs
#8,543,833
of 25,394,764 outputs
Outputs from Journal of Molecular Neuroscience
#485
of 1,643 outputs
Outputs of similar age
#28,485
of 79,668 outputs
Outputs of similar age from Journal of Molecular Neuroscience
#4
of 8 outputs
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