Glyceryl Trioleate

(rINN)

💊 Chemical information

Triolein. 1,2,3-Propanetriol tri(9-octandecenoate).
Chemical formula: C57H104O6 = 885.4.
CAS — 122-32-7.

💊 Profile

Lorenzo’s oil is a liquid containing glyceryl trierucate (a source of erucic acid) and glyceryl trioleate (a source of oleic acid), in the ratio 1 part to 4 parts respectively. It has been used with dietary modification for the treatment of adrenoleucodystrophy, a genetic disorder characterised by demyelination, adrenal cortical insufficiency, and accumulation of saturated ‘very-long-chain fatty acids’.

Adrenoleucodystrophy.

Adrenoleucodystrophy is a rare Xlinked metabolic disorder in which accumulation of saturated very-long-chain fatty acids results in diffuse and multifocal demyelination of the nervous system and adrenocortical insufficiency. The most common form usually affects children and is characterised primarily by cerebral demyelination; it is usually fatal within a few years. In the adult variant, called adrenomyeloneuropathy, demyelination of the spinal cord and peripheral neuropathy progress slowly over many years.1,2 There appears to be no effective treatment for adrenoleucodystrophy or its variants. A high dietary intake of long-chain monounsaturated fatty acids, as provided by the mixture Lorenzo’s oil (glyceryl trierucate with glyceryl trioleate), has been tried, the idea being to monopolise the specific enzyme involved in the conversion of long-chain fatty acids to very-long-chain fatty acids. Although dietary therapy with Lorenzo’s oil has reduced plasma concentrations of saturated very-long-chain fatty acids, there is no evidence that this improves or delays progression of adrenoleucodystrophy or adrenomyeloneuropathy.3-6 However, it has been suggested that these disorders may not respond to correction of the biochemical abnormality once neurological damage has occurred.5 The effectiveness of treatment before the appearance of neurological symptoms is currently being studied with some encouraging results.7 There is some evidence to suggest that the childhood form may have an immunological component, but results using immunosuppressants or immunoglobulins have been reported to be disappointing.5 Bone marrow transplants may improve symptoms but should only be tried in those with mild cerebral involvement.1 Lovastatin can also reduce plasma concentrations of very-long-chain fatty acids.8
1. van Geel BM, et al. X linked adrenoleukodystrophy: clinical presentation, diagnosis, and therapy. J Neurol Neurosurg Psychiatry 1997; 63: 4–14
2. Moser HW, et al. Adrenoleukodystrophy: new approaches to a neurodegenerative disease. JAMA 2005; 294: 3131–4
3. Aubourg P, et al. A two-year trial of oleic and erucic acids ("Lorenzo’s oil") as treatment for adrenomyeloneuropathy. N Engl J Med 1993; 329: 745–52
4. Kaplan PW, et al. Visual evoked potentials in adrenoleukodystrophy: a trial with glycerol trioleate and Lorenzo oil. Ann Neurol 1993; 34: 169–74
5. Rizzo WB. Lorenzo’s oil—hope and disappointment. N Engl J Med 1993; 329: 801–2
6. van geel BM, et al. Progression of abnormalities in adrenomyeloneuropathy and neurologically asymptomatic X-linked adrenoleukodystrophy despite treatment with "Lorenzo’s oil". J Neurol Neurosurg Psychiatry 1999; 67: 290–9
7. Moser HW, et al. Follow-up of 89 asymptomatic patients with adrenoleukodystrophy treated with Lorenzo’s oil. Arch Neurol 2005; 62: 1073–80
8. Pai GS, et al. Lovastatin therapy for X-linked adrenoleukodystrophy: clinical and biochemical observations on 12 patients. Mol Genet Metab 2000; 69: 312–22.

Adverse effects.

Thrombocytopenia has been reported in patients receiving Lorenzo’s oil, although patients are often asymptomatic.1 It is possible that giant platelets which retain most of their function are produced and that these are not counted by automatic counting procedures giving a false impression of thrombocytopenia.2 Lymphocytopenia with an increased incidence of infection has also been reported in few patients.3
1. Zinkham WH, et al. Lorenzo’s oil and thrombocytopenia in patients with adrenoleukodystrophy. N Engl J Med 1993; 328: 1126–7
2. Stöckler S, et al. Giant platelets in erucic acid therapy for adrenoleukodystrophy. Lancet 1993; 341: 1414–15
3. Unkrig CJ, et al. Lorenzo’s oil and lymphocytopenia. N Engl J Med 1994; 330: 577.

💊 Preparations

Proprietary Preparations

Ital.: GTO Oil.
Published May 08, 2019.