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Titolo:
PEROXISOMAL BETA-OXIDATION OF POLYUNSATURATED LONG-CHAIN FATTY-ACIDS IN HUMAN FIBROBLASTS - THE POLYUNSATURATED AND THE SATURATED LONG-CHAIN FATTY-ACIDS ARE RETROCONVERTED BY THE SAME ACYL-COA OXIDASE
Autore:
CHRISTENSEN E; WOLDSETH B; HAGVE TA; POLLTHE BT; WANDERS RJA; SPRECHER H; STOKKE O; CHRISTOPHERSEN BO;
Indirizzi:
NATL HOSP NORWAY,DEPT CLIN CHEM N-0027 OSLO NORWAY UNIV OSLO,RIKSHOSP,INST CLIN BIOCHEM OSLO NORWAY UNIV UTRECHT,CHILDRENS HOSP UTRECHT NETHERLANDS UNIV AMSTERDAM HOSP,DEPT PEDIAT AMSTERDAM NETHERLANDS OHIO STATE UNIV,DEPT MED BIOCHEM COLUMBUS OH 43210
Titolo Testata:
Scandinavian journal of clinical & laboratory investigation
, volume: 53, anno: 1993, supplemento:, 215
pagine: 61 - 74
SICI:
0036-5513(1993)53:<61:PBOPLF>2.0.ZU;2-6
Fonte:
ISI
Lingua:
ENG
Soggetto:
RAT-LIVER PEROXISOMES; X-LINKED ADRENOLEUKODYSTROPHY; CULTURED SKIN FIBROBLASTS; ZELLWEGER SYNDROME; ERUCIC-ACID; NEONATAL ADRENOLEUKODYSTROPHY; TRIHYDROXYCOPROSTANOYL-COA; DOCOSAHEXAENOIC ACID; DELTA-4 DESATURATION; PRENATAL-DIAGNOSIS;
Keywords:
ACYL-COA OXIDASE DEFICIENCY; DOCOSAHEAXAENOATE; DOCOSAPENTAENOATE; DOCOSATETRAENATE; ERUCATE; POLYUNSATURATED FATTY ACIDS; RETROCONVERSION; X-LINKED ADRENOLEUKODYSTROPHY; ZELLWEGER SYNDROME;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
51
Recensione:
Indirizzi per estratti:
Citazione:
E. Christensen et al., "PEROXISOMAL BETA-OXIDATION OF POLYUNSATURATED LONG-CHAIN FATTY-ACIDS IN HUMAN FIBROBLASTS - THE POLYUNSATURATED AND THE SATURATED LONG-CHAIN FATTY-ACIDS ARE RETROCONVERTED BY THE SAME ACYL-COA OXIDASE", Scandinavian journal of clinical & laboratory investigation, 53, 1993, pp. 61-74

Abstract

The metabolism of the C22 unsaturated fatty acids erucic acid (22:1(n-9)), adrenic acid (22:4(n-6)), docosapentaenoic acid (22:5(n-3)) and docosahexaenoic acid (22:6(n-3)) was studied in cultured fibroblasts from patients with acyl-CoA oxidase deficiency, the Zellweger syndrome,X-linked adrenoleukodystrophy (X-ALD) and normal controls. [3-C-14] 22:4 (n-6) and [3-C-14] 22:5 (n-3) were shortened (retroconverted) to [1-C-14] 20:4 (n-6) and [1-C-14] 20:5 (n-3), respectively, in normal and X-ALD fibroblasts. In Zellweger and acyl-CoA oxidase deficient fibroblasts these reactions were deficient. Since the retroconversion is normal in X-ALD fibroblasts peroxisomal very long chain (lignoceryl) CoAligase is probably not required for the activation of C22 unsaturatedfatty acids. The present work with fibroblasts from patients with a specific acyl-CoA oxidase deficiency, previously shown to have a deficient peroxisomal clofibrate-inducible acyl-CoA oxidase, and which accumulate 24:0 and 26:0 fatty acids, supports the view that this enzyme isresponsible for the chain-shortening of docosahexaenoic acid (22:6(n-3)), erucic acid (22:1(n-9)), docosapentaenoic acid (22:5(n-3)), and adrenic acid (22:4(n-6)) as well.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 02/07/20 alle ore 20:29:08