Article
Analysis of differential neonatal lethality in cystathionine β-synthase deficient mouse models using metabolic profiling.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology - 1 Jun 2021
Gupta Sapna, Wang Liqun, Slifker Michael J, Cai Kathy Q, Maclean Kenneth N, Wasek Brandi, Bottiglieri Teodoro, Kruger Warren D
Abstract excerpt
Cystathionine beta-synthase (CBS) is a key enzyme of the trans-sulfuration pathway that converts homocysteine to cystathionine. Loss of CBS activity due to mutation results in CBS deficiency, an inborn error of metabolism characterized by extreme elevation of plasma total homocysteine (tHcy). C57BL6 mice containing either a homozygous null mutation in the cystathionine β-synthase (Cbs-/- ) gene or an inactive...
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