PMID-sentid Pub_year Sent_text comp_official_name comp_offsetprotein_name organism prot_offset 33949005-2 2021 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). thcy 159-163 cystathionine beta-synthase Mus musculus 8-11 34528713-1 2021 Cystathionine beta-synthase (CBS) deficiency is a recessive inborn error of sulfur metabolism characterized by elevated blood levels of total homocysteine (tHcy). thcy 156-160 cystathionine beta-synthase Mus musculus 0-27 31240737-5 2019 Zinc-treated Tg-R336C Cbs -/- mice have extreme elevation in both serum total homocysteine (tHcy) and liver tHcy compared with control transgenic mice. thcy 92-96 cystathionine beta-synthase Mus musculus 22-25 31084364-1 2019 Cystathionine beta-synthase (CBS) deficiency is a recessive inborn error of metabolism characterized by extremely elevated total homocysteine (tHcy) in the blood. thcy 143-147 cystathionine beta-synthase Mus musculus 0-27 31240737-5 2019 Zinc-treated Tg-R336C Cbs -/- mice have extreme elevation in both serum total homocysteine (tHcy) and liver tHcy compared with control transgenic mice. thcy 108-112 cystathionine beta-synthase Mus musculus 22-25 27183385-5 2016 Here, we have shown that administration of PEGylated CBS into the circulation of homocystinuria model mice alters the extra- and intracellular equilibrium of sulfur amino acids, resulting in a decrease of approximately 75% in plasma total homocysteine (tHcy) and normalization of cysteine concentrations. thcy 253-257 cystathionine beta-synthase Mus musculus 53-56 24189943-4 2014 Cbs(-/-) mice fed the MRD had a 77% decrease in tHcy, 28% increase in weight, 130% increase in fat mass, 82% increase in Scd-1 expression, and 10.6% increase in bone density and entirely lacked the alopecia phenotype observed in age-matched Cbs(-/-) mice fed the RD. thcy 48-52 cystathionine beta-synthase Mus musculus 0-3 26599618-2 2016 Mutations in the CBS gene cause clinical CBS deficiency, a disease characterized by elevated plasma total homocysteine (tHcy) and methionine and decreased plasma cysteine. thcy 120-124 cystathionine beta-synthase Mus musculus 17-20 26599618-5 2016 Tg-I278T Cbs(-/-) mice have undetectable levels of CBS activity, extremely elevated plasma tHcy, modestly elevated plasma methionine, and low plasma cysteine. thcy 91-95 cystathionine beta-synthase Mus musculus 9-12 26231230-1 2016 Cystathionine beta synthase (CBS) deficiency is a recessive inborn error of metabolism characterized by elevated serum total homocysteine (tHcy). thcy 139-143 cystathionine beta-synthase Mus musculus 0-27 26231230-5 2016 Tg-I278T Cbs (-/-) mice on betaine, from the time of weaning until for 240 days of age, had a 40 % decrease in mean tHcy level and a 137 % increase in serum methionine levels. thcy 116-120 cystathionine beta-synthase Mus musculus 9-12 20036517-5 2010 Because CBS is a key regulator of tHcy, we examined CBS protein levels and found that within 3 d on the methionine-deficient diet, animals had a 50% reduction in the levels of liver CBS protein and enzyme activity. thcy 34-38 cystathionine beta-synthase Mus musculus 8-11 17537983-5 2007 The higher renal CBS activity in males (22.7 +/- 3.1 mmol cystathionine.h(-1).kg kidney(-1)) vs. females (8.4 +/- 3.4 mmol cystathionine.h(-1).kg kidney(-1), P < or = 10(-6)) in C57Bl/6J mice was associated with lower plasma tHcy levels in males vs. females, and this difference was exacerbated in Cbs+/- mice (7.7 +/- 1.9 micromol/l in males vs. 13.8 +/- 6.4 micromol/l in females, P = 0.005). thcy 228-232 cystathionine beta-synthase Mus musculus 17-20 17537983-4 2007 Renal function is known to be an important determinant of tHcy, and, in this study, we demonstrate that renal CBS expression and activity in mice diminished approximately twofold after castration, whereas ovariectomization was without effect. thcy 58-62 cystathionine beta-synthase Mus musculus 110-113