21:02 Nov 6, 2003 |
German to English translations [PRO] Medical | |||||
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| Selected response from: Martinique Local time: 17:53 | ||||
Grading comment
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Summary of answers provided | ||||
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4 -1 | MTP test |
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3 | test for mitochondrial trifunctional protein (MTP) |
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MTP test Explanation: metatarsophalangeal = abbr. MTP -------------------------------------------------- Note added at 2003-11-06 21:07:37 (GMT) -------------------------------------------------- www.apodc.com.au/AJPM/Contents/Full text/Vol37/ abstracts/Vol%2037_2_43-45abstract.pdf |
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test for mitochondrial trifunctional protein (MTP) Explanation: OR test for microsomal triglyceride transfer protein (¶MTP) Harrison's Pronciples of Internal Medicine: Abetalipoproteinemia is caused by mutations in the gene coding for the larger subunit of the microsomal triglyceride transfer protein (¶MTP). Defects in ¶MTP result in impairment of formation and secretion of VLDL in liver. This defect results in a deficiency of delivery of vitamin E to tissues, including the central and peripheral nervous system http://www.emedicine.com/ped/topic1284.htm Long-chain 3-hydroxy acyl-coenzyme A dehydrogenase (LCHAD) is 1 of 3 enzymatic activities that make up the ***trifunctional protein of the inner mitochondrial membrane. The other 2 activities of the protein are 2-enoyl coenzyme A (CoA) hydratase (LCEH) and long-chain 3-ketoacyl CoA thiolase (LCKT). The protein is an octamer composed of 4 alpha subunits that contain the LCEH and LCHAD activities, and 4 beta subunits that contain the LCKT activity. This enzyme complex metabolizes long-chain fatty acids, and the LCHAD activity is specific for compounds of C12-C16 chain length. The genes for the alpha and beta subunits have been localized to chromosome 2. Affected infants with LCHAD deficiency, which is inherited as an autosomal recessive trait, present in late infancy with acute hypoketotic hypoglycemia. These episodes typically appear for the first time after a fast, which usually occurs in the context of intercurrent illness with vomiting. Pathophysiology: The molecular defect occurs in the ***mitochondrial trifunctional protein (MTP)... P.S. These are just guesses... -------------------------------------------------- Note added at 16 hrs 34 mins (2003-11-07 13:36:14 GMT) -------------------------------------------------- Yes, this is microsomal triglyceride transfer protein. Look here: Die Konzentration zirkulierender LDL kann sowohl erniedrigt als auch erhöht sein. Bei der Abetalipoproteinämie (BassenKornzweigSyndrom) fehlt die Synthese von VLDL in der Leber, bzw. von Chylomikronen im Darm, was zur konsekutiven starken Verminderung der LDLKonzentration im Plasma führt. Ursache ist ein genetischer Mangel an ***MTP (***Mikrosomales Transferprotein***), das für die Bindung von Lipiden an das gerade ebensynthetisierte Apolipoprotein B zuständig ist. Das sich anhäufende Apolipoprotein B wird dann innerhalb der Leber bzw. Darmzelle sofort wieder abgebaut. Ursache der Hypobetalipoproteinämie ist die Produktion und Abgabe defekter ApolipoproteinBMoleküle (truncated ApoB), die zu einer extrem verkürzten Lebensdauer der LDLPartikel im Plasma führen und somit die LDLKonzentration erniedrigen. Die Abetalipoproteinämie führt in der Regel zu einer leichten Anämie und zu einem mäßig ausgeprägten Malabsorptionssyndrom, jedoch offenbar nicht zu einer wesentlich verkürzten Lebenserwartung. http://www.lipid-liga.de/inhalt/veranstaltungen_doku.htm Reference: http://www.emedicine.com/ped/topic1284.htm |
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