Literature DB >> 3741408

Detection of a homologous series of C26-C38 polyenoic fatty acids in the brain of patients without peroxisomes (Zellweger's syndrome).

A Poulos, P Sharp, H Singh, D Johnson, A Fellenberg, A Pollard.   

Abstract

The brains of patients with inherited abnormalities in peroxisomal structure and function contain greatly increased proportions of a homologous series of unique polyenoic fatty acids with carbon chain lengths ranging from 26 to 38. Based on evidence by chemical ionization and electron impact mass spectrometry before and after catalytic hydrogenation, and argentation t.l.c., these lipids have been tentatively identified as 26:5, 28:5, 30:5, 30:6, 30:7, 32:5, 32:6, 32:7, 34:5 and 34:6 fatty acids. A further two fatty acids eluting at very high temperatures from gas chromatography columns have been tentatively identified on the basis of their chemical ionization mass spectra as 36:6 and 38:6 fatty acids.

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Year:  1986        PMID: 3741408      PMCID: PMC1146726          DOI: 10.1042/bj2350607

Source DB:  PubMed          Journal:  Biochem J        ISSN: 0264-6021            Impact factor:   3.857


  16 in total

1.  A simple method for the isolation and purification of total lipides from animal tissues.

Authors:  J FOLCH; M LEES; G H SLOANE STANLEY
Journal:  J Biol Chem       Date:  1957-05       Impact factor: 5.157

Review 2.  Review: the cerebrohepatorenal syndrome of Zellweger, morphologic and metabolic aspects.

Authors:  R I Kelley
Journal:  Am J Med Genet       Date:  1983-12

3.  Assay for polyunsaturated fatty acids by argentation--thin-layer chromatography using commercial thin-layer plates.

Authors:  M Inomata; F Takaku; Y Nagai; M Saito
Journal:  Anal Biochem       Date:  1982-09-01       Impact factor: 3.365

4.  Metabolism of arachidonate in rat testis: characterization of 26-30 carbon polyenoic acids.

Authors:  W M Grogan
Journal:  Lipids       Date:  1984-05       Impact factor: 1.880

5.  The cerebrohepatorenal (Zellweger) syndrome. Increased levels and impaired degradation of very-long-chain fatty acids and their use in prenatal diagnosis.

Authors:  A E Moser; I Singh; F R Brown; G I Solish; R I Kelley; P J Benke; H W Moser
Journal:  N Engl J Med       Date:  1984-05-03       Impact factor: 91.245

6.  Adrenoleukodystrophy: impaired oxidation of very long chain fatty acids in white blood cells, cultured skin fibroblasts, and amniocytes.

Authors:  I Singh; A E Moser; H W Moser; Y Kishimoto
Journal:  Pediatr Res       Date:  1984-03       Impact factor: 3.756

7.  Degradation of unsaturated fatty acids in peroxisomes. Existence of a 2,4-dienoyl-CoA reductase pathway.

Authors:  V Dommes; C Baumgart; W H Kunau
Journal:  J Biol Chem       Date:  1981-08-25       Impact factor: 5.157

Review 8.  Supply of polyenoic fatty acids to the mammalian brain: the ease of conversion of the short-chain essential fatty acids to their longer chain polyunsaturated metabolites in liver, brain, placenta and blood.

Authors:  J M Naughton
Journal:  Int J Biochem       Date:  1981

9.  The biosynthesis of delta-9,12,15,18-tetracosatetraenoic and of delta-6,9,12,15,18-tetracosapentaenoic acids by rat testes.

Authors:  R B Bridges; J G Coniglio
Journal:  J Biol Chem       Date:  1970-01-10       Impact factor: 5.157

10.  Biosynthesis of long-chain polyenoic acids from arachidonic acid in cultures of enriched spermatocytes and spermatids from mouse testis.

Authors:  W M Grogan; E G Huth
Journal:  Lipids       Date:  1983-04       Impact factor: 1.880

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  23 in total

1.  Long-chain and very long-chain polyunsaturated fatty acids in ocular aging and age-related macular degeneration.

Authors:  Aihua Liu; James Chang; Yanhua Lin; Zhengqing Shen; Paul S Bernstein
Journal:  J Lipid Res       Date:  2010-08-05       Impact factor: 5.922

Review 2.  Retinal very long-chain PUFAs: new insights from studies on ELOVL4 protein.

Authors:  Martin-Paul Agbaga; Md Nawajes A Mandal; Robert E Anderson
Journal:  J Lipid Res       Date:  2010-03-18       Impact factor: 5.922

3.  Molecular species of phospholipids with very long chain fatty acids in skin fibroblasts of Zellweger syndrome.

Authors:  Kotaro Hama; Toru Nagai; Chiho Nishizawa; Kazutaka Ikeda; Masashi Morita; Noriko Satoh; Hiroki Nakanishi; Tsuneo Imanaka; Nobuyuki Shimozawa; Ryo Taguchi; Keizo Inoue; Kazuaki Yokoyama
Journal:  Lipids       Date:  2013-12       Impact factor: 1.880

4.  Peroxisomal integral membrane proteins in livers of patients with Zellweger syndrome, infantile Refsum's disease and X-linked adrenoleukodystrophy.

Authors:  G M Small; M J Santos; T Imanaka; A Poulos; D M Danks; H W Moser; P B Lazarow
Journal:  J Inherit Metab Dis       Date:  1988       Impact factor: 4.982

Review 5.  Very-long-chain fatty acids from lower organism.

Authors:  T Rezanka; J Cudlín; M Podojil
Journal:  Folia Microbiol (Praha)       Date:  1987       Impact factor: 2.099

6.  Synthesis of very long chain (up to 36 carbon) tetra, penta and hexaenoic fatty acids in retina.

Authors:  N P Rotstein; M I Aveldaño
Journal:  Biochem J       Date:  1988-01-01       Impact factor: 3.857

Review 7.  On the molecular etiology of decreased arachidonic (20:4n-6), docosapentaenoic (22:5n-6) and docosahexaenoic (22:6n-3) acids in Zellweger syndrome and other peroxisomal disorders.

Authors:  J P Infante; V A Huszagh
Journal:  Mol Cell Biochem       Date:  1997-03       Impact factor: 3.396

8.  Relationship between dietary supply of long-chain fatty acids and membrane composition of long- and very long chain essential fatty acids in developing rat photoreceptors.

Authors:  M Suh; A A Wierzbicki; E Lien; M T Clandinin
Journal:  Lipids       Date:  1996-01       Impact factor: 1.880

Review 9.  Very long chain fatty acids in higher animals--a review.

Authors:  A Poulos
Journal:  Lipids       Date:  1995-01       Impact factor: 1.880

10.  Deciphering mutant ELOVL4 activity in autosomal-dominant Stargardt macular dystrophy.

Authors:  Sreemathi Logan; Martin-Paul Agbaga; Michael D Chan; Nabila Kabir; Nawajes A Mandal; Richard S Brush; Robert E Anderson
Journal:  Proc Natl Acad Sci U S A       Date:  2013-03-18       Impact factor: 11.205

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