Výsledky vyhledávání - "Lori S. Greiner"

A novel transgenic mouse model of CBS-deficient homocystinuria does not incur hepatic steatosis or fibrosis and exhibits a hypercoagulative phenotype that is ameliorated by betaine treatment

Autor: Katherine H. Overdier, Gary Brodsky, Milan Elleder, Rima Rozen, Kenneth N. Maclean, Viktor Kožich, Linda S. Crnic, Lori S. Greiner, Hua Jiang, Lynne Meltesen, Renata Collard, Jakub Krijt, Jakub Sikora, David Patterson, Sally P. Stabler, Robert H. Allen, Eva Kraus, Jan P. Kraus

Publikováno v: Molecular Genetics and Metabolism

Cystathionine beta-synthase (CBS) catalyzes the condensation of homocysteine (Hcy) and serine to cystathionine, which is then hydrolyzed to cysteine by cystathionine gamma-lyase. Inactivation of CBS results in CBS-deficient homocystinuria more common

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::dfa311a214521f5b818ba15f40c7edef

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Cystathionine protects against endoplasmic reticulum stress-induced lipid accumulation, tissue injury, and apoptotic cell death

Autor: Richard C. Austin, Lori S. Greiner, Sudesh K. Sood, Hua Jiang, Neil E. Markham, Vivek Balasubramaniam, Kenneth N. Maclean, Jeffrey R. Evans, Sally P. Stabler, Robert H. Allen, Šárka Lhoták

Publikováno v: The Journal of biological chemistry. 287(38)

Cystathionine (R-S-(2-amino-2-carboxyethyl)-l-homocysteine) is a non-proteinogenic thioether containing amino acid. In mammals, cystathionine is formed as an intermediate of the transsulfuration pathway by the condensation of serine and homocysteine

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::6a353c5fe87dcc5d65eb7a08635c28b5
https://pubmed.ncbi.nlm.nih.gov/22854956

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Long-term betaine therapy in a murine model of cystathionine beta-synthase deficient homocystinuria: decreased efficacy over time reveals a significant threshold effect between elevated homocysteine and thrombotic risk

Autor: Kenneth N. Maclean, Hua Jiang, Robert H. Allen, Sally P. Stabler, Lori S. Greiner

Publikováno v: Molecular genetics and metabolism. 105(3)

Classical homocystinuria (HCU) is caused by deficiency of cystathionine β-synthase and is characterized by connective tissue disturbances, mental retardation and cardiovascular disease. Treatment for pyridoxine non-responsive HCU typically involves

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::7d0a93ac9122700cd5ac88df0b2ba556
https://pubmed.ncbi.nlm.nih.gov/22192524

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