Biomaterial Database

Chorea-acanthocytosis: abnormal composition of covalently bound fatty acids of erythrocyte membrane proteins. 1991

T Sakai, and Y Antoku, and H Iwashita, and I Goto, and K Nagamatsu, and H Shii

Department of Neurology, National Chikugo Hospital, Fukuoka, Japan.

Phospholipid class, peak profile of each phospholipid class, loosely bound fatty acids, covalently (tightly) bound fatty acids of the erythrocyte membranes, and plasma fatty acids were investigated using high-performance liquid chromatography in six patients with chorea-acanthocytosis and 14 age- and sex-matched normal control subjects. Additionally, six patients with Huntington's disease were included as disease control subjects in the study of covalently bound fatty acids. Study of covalently (tightly) bound fatty acids in erythrocyte membrane proteins after alkaline hydrolysis, hitherto undescribed in chorea-acanthocytosis, revealed that palmitic acid (C16:0) was significantly increased and stearic acid (C18:0) was decreased in the patients with chorea-acanthocytosis. Analyses for total covalently bound fatty acids disclosed that palmitic and docosahexaenoic (C22:6) acids were increased and stearic acid was decreased in chorea-acanthocytosis. Phospholipid class (phosphatidylcholine, phosphatidylethanolamine, sphingomyelin, and phosphatidylserine) and peak profile of each phospholipid class from the erythrocyte membranes did not differ between the patients with chorea-acanthocytosis and the control subjects. Of the loosely bound fatty acids, linoleic acid (C18:2) was significantly decreased in those with chorea-acanthocytosis, which seemed to be nonspecific.

UI	MeSH Term	Description	Entries
D008041	Linoleic Acids	Eighteen-carbon essential fatty acids that contain two double bonds.	Acids, Linoleic
D008297	Male		Males
D008563	Membrane Lipids	Lipids, predominantly phospholipids, cholesterol and small amounts of glycolipids found in membranes including cellular and intracellular membranes. These lipids may be arranged in bilayers in the membranes with integral proteins between the layers and peripheral proteins attached to the outside. Membrane lipids are required for active transport, several enzymatic activities and membrane formation.	Cell Membrane Lipid,Cell Membrane Lipids,Membrane Lipid,Lipid, Cell Membrane,Lipid, Membrane,Lipids, Cell Membrane,Lipids, Membrane,Membrane Lipid, Cell,Membrane Lipids, Cell
D008565	Membrane Proteins	Proteins which are found in membranes including cellular and intracellular membranes. They consist of two types, peripheral and integral proteins. They include most membrane-associated enzymes, antigenic proteins, transport proteins, and drug, hormone, and lectin receptors.	Cell Membrane Protein,Cell Membrane Proteins,Cell Surface Protein,Cell Surface Proteins,Integral Membrane Proteins,Membrane-Associated Protein,Surface Protein,Surface Proteins,Integral Membrane Protein,Membrane Protein,Membrane-Associated Proteins,Membrane Associated Protein,Membrane Associated Proteins,Membrane Protein, Cell,Membrane Protein, Integral,Membrane Proteins, Integral,Protein, Cell Membrane,Protein, Cell Surface,Protein, Integral Membrane,Protein, Membrane,Protein, Membrane-Associated,Protein, Surface,Proteins, Cell Membrane,Proteins, Cell Surface,Proteins, Integral Membrane,Proteins, Membrane,Proteins, Membrane-Associated,Proteins, Surface,Surface Protein, Cell
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D010169	Palmitic Acids	A group of 16-carbon fatty acids that contain no double bonds.	Acids, Palmitic
D010743	Phospholipids	Lipids containing one or more phosphate groups, particularly those derived from either glycerol (phosphoglycerides see GLYCEROPHOSPHOLIPIDS) or sphingosine (SPHINGOLIPIDS). They are polar lipids that are of great importance for the structure and function of cell membranes and are the most abundant of membrane lipids, although not stored in large amounts in the system.	Phosphatides,Phospholipid
D002819	Chorea	Involuntary, forcible, rapid, jerky movements that may be subtle or become confluent, markedly altering normal patterns of movement. Hypotonia and pendular reflexes are often associated. Conditions which feature recurrent or persistent episodes of chorea as a primary manifestation of disease are referred to as CHOREATIC DISORDERS. Chorea is also a frequent manifestation of BASAL GANGLIA DISEASES.	Choreatic Disorders,Choreiform Movement,Chorea Disorders,Chorea Syndromes,Chorea, Benign Hereditary,Chorea, Chronic Progressive,Chorea, Rheumatic,Chorea, Senile,Chorea, Sydenham,Choreatic Syndromes,Choreic Movement,Dyskinesias, Paroxysmal,Hereditary Chorea,Hereditary Progressive Chorea Without Dementia,Paroxysmal Dyskinesias,Rheumatic Chorea,Senile Chorea,St. Vitus's Dance,Sydenham Chorea,Sydenham's Chorea,Benign Hereditary Chorea,Benign Hereditary Choreas,Chorea Disorder,Chorea Syndrome,Chorea, Hereditary,Chorea, Sydenham's,Choreas,Choreas, Benign Hereditary,Choreas, Chronic Progressive,Choreas, Hereditary,Choreas, Rheumatic,Choreas, Senile,Choreas, Sydenham,Choreatic Disorder,Choreatic Syndrome,Choreic Movements,Choreiform Movements,Chronic Progressive Chorea,Chronic Progressive Choreas,Disorder, Chorea,Disorder, Choreatic,Disorders, Chorea,Disorders, Choreatic,Dyskinesia, Paroxysmal,Hereditary Chorea, Benign,Hereditary Choreas,Hereditary Choreas, Benign,Movement, Choreic,Movement, Choreiform,Movements, Choreic,Movements, Choreiform,Paroxysmal Dyskinesia,Progressive Chorea, Chronic,Progressive Choreas, Chronic,Rheumatic Choreas,Senile Choreas,St. Vitus Dance,St. Vitus's Dances,St. Vituss Dance,Sydenham Choreas,Sydenhams Chorea,Syndrome, Chorea,Syndrome, Choreatic,Syndromes, Chorea,Syndromes, Choreatic
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