Cystathionine beta-synthase null homocystinuric mice fail to exhibit altered hemostasis or lowering of plasma homocysteine in response to betaine treatment

Kód výsledku v IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11110%2F10%3A7704" target="_blank" >RIV/00216208:11110/10:7704 - isvavai.cz</a>

Nalezeny alternativní kódy

RIV/00064165:_____/10:7704

Výsledek na webu

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DOI - Digital Object Identifier

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Jazyk výsledku

angličtina

Název v původním jazyce

Cystathionine beta-synthase null homocystinuric mice fail to exhibit altered hemostasis or lowering of plasma homocysteine in response to betaine treatment

Popis výsledku v původním jazyce

Cystathionine beta-synthase knockout mouse exhibits severe liver injury that is not found in the human cystathionine beta-synthase deficiency (homocystinuria) disease. The hepatopathy observed in this model may offer an explanation for the failure of cbs(-/-) mice to respond to betaine or exhibit a hypercoagulative phenotype. It is concluded that although this model provides useful data on the biochemical sequelae of classical homocystinuria, it does not successfully recapitulate a number of importantfeatures of the human disease and its use for studying mechanisms in homocystinuria should be treated with caution as the hepatopathy produces changes which could influence the results.

Název v anglickém jazyce

Cystathionine beta-synthase null homocystinuric mice fail to exhibit altered hemostasis or lowering of plasma homocysteine in response to betaine treatment

Popis výsledku anglicky

Cystathionine beta-synthase knockout mouse exhibits severe liver injury that is not found in the human cystathionine beta-synthase deficiency (homocystinuria) disease. The hepatopathy observed in this model may offer an explanation for the failure of cbs(-/-) mice to respond to betaine or exhibit a hypercoagulative phenotype. It is concluded that although this model provides useful data on the biochemical sequelae of classical homocystinuria, it does not successfully recapitulate a number of importantfeatures of the human disease and its use for studying mechanisms in homocystinuria should be treated with caution as the hepatopathy produces changes which could influence the results.

Druh

J_x - Nezařazeno - Článek v odborném periodiku (Jimp, Jsc a Jost)

CEP obor

EB - Genetika a molekulární biologie

OECD FORD obor

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Projekt

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Návaznosti

Z - Vyzkumny zamer (s odkazem do CEZ)

Rok uplatnění

2010

Kód důvěrnosti údajů

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Název periodika

Molecular Genetics and Metabolism

ISSN

1096-7192

e-ISSN

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Svazek periodika

101

Číslo periodika v rámci svazku

2.III

Stát vydavatele periodika

US - Spojené státy americké

Počet stran výsledku

9

Strana od-do

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Kód UT WoS článku

000283609600013

EID výsledku v databázi Scopus

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