BIOMAT Database Logo BIOMAT Database Logo

Analysis of very long-chain fatty acids and plasmalogen in the erythrocyte membrane: a simple method for the detection of peroxisomal disorders and discrimination between adrenoleukodystrophy and Zellweger syndrome. 1990

K Tanaka, and K Nishizawa, and H Yamamoto, and T Naruto, and E Izeki, and T Taga, and M Shimada, and Y Saeki
Department of Pediatrics, Nagahama Red Cross Hospital, Japan.

We analyzed the sphingomyelin very long-chain fatty acids (VLCFAs) and phosphatidylethanolamine (PE) plasmalogen contents of the erythrocyte membrane in patients suffering from peroxisomal disorders. In a patient with Zellweger syndrome, both a decrease in the PE plasmalogen content and an increase in the sphingomyelin VLCFAs content of the erythrocyte membrane were noted. In patients with adrenoleukodystrophy, however, there was no decrease in PE plasmalogen, although the sphingomyelin VLCFAs content of the membrane was significantly increased in comparison with control values. Analyses of both sphingomyelin VLCFAs and PE plasmalogen were carried out simultaneously, using both the same process and the same sample.

UI MeSH Term Description Entries
D008830 Microbodies Electron-dense cytoplasmic particles bounded by a single membrane, such as PEROXISOMES; GLYOXYSOMES; and glycosomes. Glycosomes,Glycosome,Microbody
D010714 Phosphatidylethanolamines Derivatives of phosphatidic acids in which the phosphoric acid is bound in ester linkage to an ethanolamine moiety. Complete hydrolysis yields 1 mole of glycerol, phosphoric acid and ethanolamine and 2 moles of fatty acids. Cephalin,Cephalins,Ethanolamine Phosphoglyceride,Ethanolamine Phosphoglycerides,Ethanolamineglycerophospholipids,Phosphoglyceride, Ethanolamine,Phosphoglycerides, Ethanolamine
D010955 Plasmalogens GLYCEROPHOSPHOLIPIDS in which one of the two acyl chains is attached to glycerol with an ether alkenyl linkage instead of an ester as with the other glycerophospholipids. Phosphatidal Compounds,Plasmalogen,Alkenyl Ether Phospholipids,Compounds, Phosphatidal,Ether Phospholipids, Alkenyl,Phospholipids, Alkenyl Ether
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D004910 Erythrocyte Membrane The semi-permeable outer structure of a red blood cell. It is known as a red cell 'ghost' after HEMOLYSIS. Erythrocyte Ghost,Red Cell Cytoskeleton,Red Cell Ghost,Erythrocyte Cytoskeleton,Cytoskeleton, Erythrocyte,Cytoskeleton, Red Cell,Erythrocyte Cytoskeletons,Erythrocyte Ghosts,Erythrocyte Membranes,Ghost, Erythrocyte,Ghost, Red Cell,Membrane, Erythrocyte,Red Cell Cytoskeletons,Red Cell Ghosts
D005227 Fatty Acids Organic, monobasic acids derived from hydrocarbons by the equivalent of oxidation of a methyl group to an alcohol, aldehyde, and then acid. Fatty acids are saturated and unsaturated (FATTY ACIDS, UNSATURATED). (Grant & Hackh's Chemical Dictionary, 5th ed) Aliphatic Acid,Esterified Fatty Acid,Fatty Acid,Fatty Acids, Esterified,Fatty Acids, Saturated,Saturated Fatty Acid,Aliphatic Acids,Acid, Aliphatic,Acid, Esterified Fatty,Acid, Saturated Fatty,Esterified Fatty Acids,Fatty Acid, Esterified,Fatty Acid, Saturated,Saturated Fatty Acids
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000326 Adrenoleukodystrophy An X-linked recessive disorder characterized by the accumulation of saturated very long chain fatty acids in the LYSOSOMES of ADRENAL CORTEX and the white matter of CENTRAL NERVOUS SYSTEM. This disease occurs almost exclusively in the males. Clinical features include the childhood onset of ATAXIA; NEUROBEHAVIORAL MANIFESTATIONS; HYPERPIGMENTATION; ADRENAL INSUFFICIENCY; SEIZURES; MUSCLE SPASTICITY; and DEMENTIA. The slowly progressive adult form is called adrenomyeloneuropathy. The defective gene ABCD1 is located at Xq28, and encodes the adrenoleukodystrophy protein (ATP-BINDING CASSETTE TRANSPORTERS). Adrenomyeloneuropathy,Schilder-Addison Complex,X-Linked Adrenoleukodystrophy,ALD (Adrenoleukodystrophy),Addison Disease and Cerebral Sclerosis,Bronze Schilder Disease,Melanodermic Leukodystrophy,Siemerling-Creutzfeldt Disease,X-ALD,X-ALD (X-Linked Adrenoleukodystrophy),Adrenoleukodystrophy, X-Linked,Leukodystrophies, Melanodermic,Leukodystrophy, Melanodermic,Schilder Addison Complex,Siemerling Creutzfeldt Disease,X ALD,X ALD (X Linked Adrenoleukodystrophy),X Linked Adrenoleukodystrophy
D013109 Sphingomyelins A class of sphingolipids found largely in the brain and other nervous tissue. They contain phosphocholine or phosphoethanolamine as their polar head group so therefore are the only sphingolipids classified as PHOSPHOLIPIDS. Sphingomyelin

Related Publications

K Tanaka, and K Nishizawa, and H Yamamoto, and T Naruto, and E Izeki, and T Taga, and M Shimada, and Y Saeki
Very long chain fatty acids in genetic peroxisomal disease fibroblasts: differences between the cerebro-hepato-renal (Zellweger) syndrome and adrenoleukodystrophy variants.
November 1986, Clinica chimica acta; international journal of clinical chemistry,
K Tanaka, and K Nishizawa, and H Yamamoto, and T Naruto, and E Izeki, and T Taga, and M Shimada, and Y Saeki
Adrenoleukodystrophy: abnormality of very long-chain fatty acids in erythrocyte membrane phospholipids.
November 1984, Neurology,
K Tanaka, and K Nishizawa, and H Yamamoto, and T Naruto, and E Izeki, and T Taga, and M Shimada, and Y Saeki
Peroxisomal very long-chain fatty acid beta-oxidation in human skin fibroblasts: activity in Zellweger syndrome and other peroxisomal disorders.
July 1987, Clinica chimica acta; international journal of clinical chemistry,
K Tanaka, and K Nishizawa, and H Yamamoto, and T Naruto, and E Izeki, and T Taga, and M Shimada, and Y Saeki
Cerebro-hepato-renal (Zellweger) syndrome and neonatal adrenoleukodystrophy: similarities in phenotype and accumulation of very long chain fatty acids.
December 1982, The Johns Hopkins medical journal,
K Tanaka, and K Nishizawa, and H Yamamoto, and T Naruto, and E Izeki, and T Taga, and M Shimada, and Y Saeki
Gas chromatography/mass spectrometry analysis of very long chain fatty acids, docosahexaenoic acid, phytanic acid and plasmalogen for the screening of peroxisomal disorders.
October 2003, Brain & development,
K Tanaka, and K Nishizawa, and H Yamamoto, and T Naruto, and E Izeki, and T Taga, and M Shimada, and Y Saeki
[Neuropathology of peroxisomal disorders; Zellweger syndrome and neonatal adrenoleukodystrophy].
March 1992, No to hattatsu = Brain and development,
K Tanaka, and K Nishizawa, and H Yamamoto, and T Naruto, and E Izeki, and T Taga, and M Shimada, and Y Saeki
Peroxisomal disorders: complementation analysis using beta-oxidation of very long chain fatty acids.
October 1990, Biochemical and biophysical research communications,
K Tanaka, and K Nishizawa, and H Yamamoto, and T Naruto, and E Izeki, and T Taga, and M Shimada, and Y Saeki
Peroxisomal fatty acid beta-oxidation in relation to the accumulation of very long chain fatty acids in cultured skin fibroblasts from patients with Zellweger syndrome and other peroxisomal disorders.
December 1987, The Journal of clinical investigation,
K Tanaka, and K Nishizawa, and H Yamamoto, and T Naruto, and E Izeki, and T Taga, and M Shimada, and Y Saeki
X-linked adrenoleukodystrophy: defective peroxisomal oxidation of very long chain fatty acids but not of very long chain fatty acyl-CoA esters.
June 1987, Clinica chimica acta; international journal of clinical chemistry,
K Tanaka, and K Nishizawa, and H Yamamoto, and T Naruto, and E Izeki, and T Taga, and M Shimada, and Y Saeki
Phytanic acid and very long chain fatty acids in genetic peroxisomal disorders.
May 1989, Journal of clinical chemistry and clinical biochemistry. Zeitschrift fur klinische Chemie und klinische Biochemie,
Copied contents to your clipboard!