English Journal
- Plasmalogen levels in Gaucher disease.
- Moraitou M, Dimitriou E, Zafeiriou D, Reppa C, Marinakis T, Sarafidou J, Michelakakis H.SourceDepartment of Enzymology and Cellular Function, Institute of Child Health, Athens 11527, Greece.
- Blood cells, molecules & diseases.Blood Cells Mol Dis.2008 Sep-Oct;41(2):196-9. doi: 10.1016/j.bcmd.2008.03.007. Epub 2008 May 23.
- Plasmalogens represent a unique type of phospholipids characterized by the presence of a vinyl-ether bond at the sn-1 position of the glycerol backbone. Peroxisomes are essential in their biosynthesis. Their suggested functions include protection against oxidative stress, participation in signal tra
- PMID 18501647
- Recent developments in the investigation of inherited metabolic disorders using cultured human cells.
- Roe CR, Roe DS.SourceKimberly H. Courtwright & Joseph W. Summers Institute of Metabolic Disease, Baylor University Medical Center, Dallas, Texas, 75226, USA. cr.roe@baylordallas.edu
- Molecular genetics and metabolism.Mol Genet Metab.1999 Oct;68(2):243-57.
- Thepurpose of this paper is to share experience with our systems and review recent "in vitro" methods using intact cells (fibroblasts, amniocytes) in which entire metabolic pathways can be probed for inherited metabolic defects reflected by elevations of intermediates determined by tandem mass spect
- PMID 10527676
- Presence of plasma branched-chain fatty acids in multineuronal degeneration, hepatosplenomegaly and adrenocortical insufficiency.
- Yao JK, Jardine I, Dyck PJ.AbstractWe have previously reported a unique disorder in two brothers with multi-system neuronal degeneration, hepatosplenomegaly and adrenocortical deficiency. The clinical features were different from Refsum's disease. Biochemical analysis suggested that a metabolic defect of the omega 6 polyenoic fatty acid pathway may be involved. In the present study, were have further identified by gas chromatography-mass spectrometry two branched-chain fatty acids, phytanate and pristanate, in these two patients' plasma. This small, but unequivocally elevated elevated amount of branched-chain fatty acids were primarily localized in the triacylglycerols of plasma low density lipoprotein. Such branched-chain fatty acids were not detected in skin, liver and sural nerve samples. These two cases may represent an alternative metabolic error to that found in Refsum's disease leading to phytanate accumulation.
- Journal of the neurological sciences.J Neurol Sci.1982 Aug;55(2):185-95.
- We have previously reported a unique disorder in two brothers with multi-system neuronal degeneration, hepatosplenomegaly and adrenocortical deficiency. The clinical features were different from Refsum's disease. Biochemical analysis suggested that a metabolic defect of the omega 6 polyenoic fatty a
- PMID 7131031
Japanese Journal
- 微生物によるイソプレノイド炭化水素pristaneの代謝〔イソプレノイド炭化水素の微生物酸化-4-〕
- 中島 健二,佐藤 昭雄
- 日本農芸化学会誌 57(4), 299-305, 1983
- … Results on monoterminal oxidation products, pristanol, pristanic acid, pristyl pristanate and pristyl aldehyde have been reported in our previous paper. …
- NAID 130001229241
- Microbial oxidation of isoprenoid hydrocarbons. I. Microbial oxidation of the isoprenoid alkane pristane.
- NAKAJIMA Kenji,SATO Akio,MISONO Terunobu,IIDA Takeo,NAGAYASU Kozo
- Agricultural and Biological Chemistry 38(10), 1859-1865, 1974
- … Silica gel chromatography of the ethyl ether extract from the culture fluid yielded pristanol and pristanic acid as major products and pristyl pristanate and pristyl aldehyde as minor products. …
- NAID 130003524420
Related Links
- Pristanate Blood Measured with requests for very long chain fatty acids for peroxisomal disorders Pink Cap – EDTA tube Reference range Less than 1 yr 0 - 1 µmol/L 1 - 10 years 0 - 2 µmol/L 10+ years 0 -2 µmol/L Referred Test ...
- Unsaturated Fatty Acids Branched Fatty Acids 各メーカー製品の比較検討がしやすい、バイオの買物.comが提供するライフサイエンス主要メーカー20社の20万製品を掲載した メーカー横断カタログ。
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