| phytanic acid | <chemical> 3,7,11,15-tetramethylhexadecanoic acid. A 20-carbon branched chain fatty acid. In phytanic acid storage disease (refsum disease) this lipid may comprise as much as 30% of the total fatty acids of the plasma. This is due to a phytanic acid alpha-hydroxylase deficiency. Chemical name: Hexadecanoic acid, 3,7,11,15-tetramethyl- (12 Dec 1998) |
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| phytanic acid alpha-oxidase | <enzyme> May be the genetic defect in refsum's disease Registry number: EC 1.14.99.- Synonym: phytanic acid alpha-hydroxylase (26 Jun 1999) |
| phytanic acid storage disease | A genetic disorder of the fatty acid phytanic acid which accumulates and causes a number of progressive problems including polyneuritis (inflammation of numerous nerves), diminishing vision (due to retinitis pigmentosa), and wobbliness (ataxia) caused by damage to the cerebellar portion of the brain (cerebellar ataxia). (12 Dec 1998) |
| disease, phytanic acid storage | A genetic disorder of the fatty acid phytanic acid which accumulates and causes a number of progressive problems including polyneuritis (inflammation of numerous nerves), diminishing vision (due to retinitis pigmentosa), and wobbliness (ataxia) caused by damage to the cerebellar portion of the brain (cerebellar ataxia).(refsum's disease) (12 Dec 1998) |
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Synonyms : Acid, Phytanic
| phytanic acid |
an unusual 20-carbon branched-chain fatty acid occurring at high levels in dairy products and ruminant fats and accumulated in tissues of patients with Refsum's disease and several peroxisomal disorders.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| phytanic acid α-hydroxylase |
an enzyme that catalyzes the alpha oxidation of phytanic acid, hydroxylating its alpha carbon; the reaction requires molecular oxygen and is stimulated by NADPH and by ferric iron. Deficiency of the enzyme, an autosomal recessive trait, causes Refsum's disease.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| phytanic acid storage d. |
1. Refsum's d. 2. any of several disorders characterized by abnormal accumulation of phytanic acid in tissues.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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제품명 |
판매사 |
보험코드 | 성분/함량 | 구분/보험급여 |
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제품명 |
판매사 |
보험코드 | 성분/함량 | 구분/보험급여 |
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