TY - JOUR
T1 - Plasma and red blood cell fatty acids in peroxisomal disorders
AU - Moser, Ann B.
AU - Jones, David S.
AU - Raymond, Gerald V.
AU - Moser, Hugo W.
N1 - Funding Information:
Supported in part by grants FDR 001289 and 000685 from the Food and Drug Administration, and RR 00052 General Clinical Research Center from the National Institutes of Health. We thank the Martek Biosciences Corporation for the supply of the DHA and AA preparations (DHASCO and ARASCO).
PY - 1999
Y1 - 1999
N2 - The demonstration of abnormal levels of fatty acids or plasmalogens in plasma or red blood cells is key to the diagnosis of peroxisomal disorders. We report the levels of 62 fatty acids and plasmalogens in patients with X- linked adrenoleukodystrophy (X-ALD), Zellweger syndrome (ZS), neonatal adrenoleukodystrophy (NALD), and infantile Refsum disease (IRD), both at baseline and after dietary interventions. 'Lorenzo's Oil' therapy in X-ALD normalizes the levels of saturated very long chain fatty acids in plasma, but leads to reduced levels of omega 6 and other omega 3 fatty acids, and requires monitoring and appropriate dietary supplements. Patients with ZS, NALD and IRD have reduced levels of docosahexaenoic acid (DHA) and arachidonic acid (AA) which can he normalized by the oral administration of microencapsulated DHA and AA.
AB - The demonstration of abnormal levels of fatty acids or plasmalogens in plasma or red blood cells is key to the diagnosis of peroxisomal disorders. We report the levels of 62 fatty acids and plasmalogens in patients with X- linked adrenoleukodystrophy (X-ALD), Zellweger syndrome (ZS), neonatal adrenoleukodystrophy (NALD), and infantile Refsum disease (IRD), both at baseline and after dietary interventions. 'Lorenzo's Oil' therapy in X-ALD normalizes the levels of saturated very long chain fatty acids in plasma, but leads to reduced levels of omega 6 and other omega 3 fatty acids, and requires monitoring and appropriate dietary supplements. Patients with ZS, NALD and IRD have reduced levels of docosahexaenoic acid (DHA) and arachidonic acid (AA) which can he normalized by the oral administration of microencapsulated DHA and AA.
UR - https://www.scopus.com/pages/publications/0032935287
UR - https://www.scopus.com/inward/citedby.url?scp=0032935287&partnerID=8YFLogxK
U2 - 10.1023/A:1022549618333
DO - 10.1023/A:1022549618333
M3 - Article
C2 - 9972864
AN - SCOPUS:0032935287
SN - 0364-3190
VL - 24
SP - 187
EP - 197
JO - Neurochemical Research
JF - Neurochemical Research
IS - 2
ER -