کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
8302430 | 1537731 | 2014 | 10 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Very-long-chain polyunsaturated fatty acids accumulate in phosphatidylcholine of fibroblasts from patients with Zellweger syndrome and acyl-CoA oxidase1 deficiency
ترجمه فارسی عنوان
اسیدهای چرب اشباع نشده چربی بسیار طولانی در فسفاتیدیل کولین فیبروبلاست ها از بیماران مبتلا به سندرم زلله و کمبود آسیل کاکائو اکسیداز
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کلمات کلیدی
plasmenylethanolamineABCD1d-bifunctional proteinPtdChoPBDPlsEtnAOXSFAMUFALC-ESI-MS/MS - LC-ESI-MS / MSPtdEtn - PtDEtnsiRNA - siRNAperoxisome biogenesis disorder - اختلال بیوژنز پراکسسیومArachidonic acid - اسید آراشیدونیکOleic acid - اسید اولئیکdocosahexaenoic acid - اسید داکوزاگزوائونیکsaturated fatty acid - اسید چرب اشباع شدهmonounsaturated fatty acid - اسید چرب غیر اشباعPolyunsaturated fatty acid - اسید چرب غیر اشباعPUFA - اسید چرب چند غیراشباعCho - برایChinese Hamster Ovary - تخمدان هامستر چینیCNS - دستگاه عصبی مرکزیDHA - دوکوساهگزائنوئیک اسیدcentral nervous system - سیستم عصبی مرکزیphosphatidylcholine - فسفاتیدیل کولینphosphatidylethanolamine - فسفاتیدیلتانولامینComplementation group - گروه تکمیلی
موضوعات مرتبط
علوم زیستی و بیوفناوری
بیوشیمی، ژنتیک و زیست شناسی مولکولی
زیست شیمی
چکیده انگلیسی
Peroxisomes are subcellular organelles that function in multiple anabolic and catabolic processes, including β-oxidation of very-long-chain fatty acids (VLCFA) and biosynthesis of ether phospholipids. Peroxisomal disorders caused by defects in peroxisome biogenesis or peroxisomal β-oxidation manifest as severe neural disorders of the central nervous system. Abnormal peroxisomal metabolism is thought to be responsible for the clinical symptoms of these diseases, but their molecular pathogenesis remains to be elucidated. We performed lipidomic analysis to identify aberrant metabolites in fibroblasts from patients with Zellweger syndrome (ZS), acyl-CoA oxidase1 (AOx) deficiency, D-bifunctional protein (D-BP) and X-linked adrenoleukodystrophy (X-ALD), as well as in peroxisome-deficient Chinese hamster ovary cell mutants. In cells deficient in peroxisomal biogenesis, plasmenylethanolamine was remarkably reduced and phosphatidylethanolamine was increased. Marked accumulation of very-long-chain saturated fatty acid and monounsaturated fatty acids in phosphatidylcholine was observed in all mutant cells. Very-long-chain polyunsaturated fatty acid (VLC-PUFA) levels were significantly elevated, whilst phospholipids containing docosahexaenoic acid (DHA, C22:6n-3) were reduced in fibroblasts from patients with ZS, AOx deficiency, and D-BP deficiency, but not in fibroblasts from an X-ALD patient. Because patients with AOx deficiency suffer from more severe symptoms than those with X-ALD, accumulation of VLC-PUFA and/or reduction of DHA may be associated with the severity of peroxisomal diseases.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids - Volume 1841, Issue 4, April 2014, Pages 610-619
Journal: Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids - Volume 1841, Issue 4, April 2014, Pages 610-619
نویسندگان
Yuichi Abe, Masanori Honsho, Hiroki Nakanishi, Ryo Taguchi, Yukio Fujiki,