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MTP

English translation: test for mitochondrial trifunctional protein (MTP)

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21:02 Nov 6, 2003
German to English translations [PRO]
Medical
German term or phrase: MTP
Pharmaceutical - Lab Tests

MTP Test

MTP Position

Menge

Labor

Prod. Nr.

Bay Nr.

Formel

Struktur
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English translation: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

Selected response from:

Martinique
Local time: 04:41
Grading comment
4 KudoZ points were awarded for this answer

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Summary of answers provided
3test for mitochondrial trifunctional protein (MTP)
Martinique
4 -1MTP test
Kathi Stock


  

Answers


4 mins   confidence: Answerer confidence 4/5Answerer confidence 4/5 peer agreement (net): -1
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

Kathi Stock
United States
Local time: 20:41
Native speaker of: Native in GermanGerman
PRO pts in pair: 789

Peer comments on this answer (and responses from the answerer)
disagree  Martinique: Sorry, but metatarsophalangeal has nothing to do with laboratory tests
16 hrs
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14 hrs   confidence: Answerer confidence 3/5Answerer confidence 3/5
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
Martinique
Local time: 04:41
Native speaker of: Native in RussianRussian
PRO pts in pair: 32
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