PMID-sentid Pub_year Sent_text comp_official_name comp_offsetprotein_name organism prot_offset 30979808-1 2019 Carbamoyl phosphate synthetase-1 (CPS1) is the major mitochondrial urea cycle enzyme in hepatocytes. Urea 67-71 carbamoyl-phosphate synthetase 1 Mus musculus 0-32 30979808-1 2019 Carbamoyl phosphate synthetase-1 (CPS1) is the major mitochondrial urea cycle enzyme in hepatocytes. Urea 67-71 carbamoyl-phosphate synthetase 1 Mus musculus 34-38 30865431-6 2019 Like PFOA, 10 mg/kg/d of PFO4DA decreased the urea cycle-related enzyme protein levels (e.g., carbamoyl phosphate synthetase 1) and serum ammonia content in a dose-dependent manner. Urea 46-50 carbamoyl-phosphate synthetase 1 Mus musculus 94-126 29801986-1 2018 Carbamoyl phosphate synthetase 1 (CPS1) is a urea cycle enzyme that forms carbamoyl phosphate from bicarbonate, ammonia and ATP. Urea 45-49 carbamoyl-phosphate synthetase 1 Mus musculus 0-32 29801986-1 2018 Carbamoyl phosphate synthetase 1 (CPS1) is a urea cycle enzyme that forms carbamoyl phosphate from bicarbonate, ammonia and ATP. Urea 45-49 carbamoyl-phosphate synthetase 1 Mus musculus 34-38 30549841-6 2017 Furthermore, the mRNA expression levels of carbamoyl phosphate synthetase 1 (Cpsl) and arginase I (Argl), in the urea cycle, were increased by OM-X feeding. Urea 113-117 carbamoyl-phosphate synthetase 1 Mus musculus 43-75 20097174-8 2010 Because ammonia generated during fasting is toxic, SIRT5 protein might play a protective role by converting ammonia to non-toxic urea through deacetylation and activation of CPS1. Urea 129-133 carbamoyl-phosphate synthetase 1 Mus musculus 174-178 26075008-10 2015 Compared with RC, HFD repressed the mRNA and protein expression of Cps1, encoding the rate-limiting enzyme of the urea cycle. Urea 114-118 carbamoyl-phosphate synthetase 1 Mus musculus 67-71 26075008-12 2015 Expression of other urea cycle genes was also decreased by HFD relative to RC and again restored by NOB to varying degrees, which, in conjunction with Cps1 promoter reporter analysis, suggested a C/EBP-dependent mechanism for the co-induction of urea cycle genes by NOB. Urea 20-24 carbamoyl-phosphate synthetase 1 Mus musculus 151-155 22402285-12 2012 Since CPS1 is responsible for ammonia removal in the urea cycle, nitration of CPS1 with attenuated function might be involved in some diseases and drug-induced toxicities associated with mitochondrial dysfunction. Urea 53-57 carbamoyl-phosphate synthetase 1 Mus musculus 6-10 22402285-12 2012 Since CPS1 is responsible for ammonia removal in the urea cycle, nitration of CPS1 with attenuated function might be involved in some diseases and drug-induced toxicities associated with mitochondrial dysfunction. Urea 53-57 carbamoyl-phosphate synthetase 1 Mus musculus 78-82 24703693-5 2014 We validated carbamoyl phosphate synthase 1 (CPS1), the rate-limiting enzyme in urea cycle, as a glutarylated protein and demonstrated that CPS1 is targeted by SIRT5 for deglutarylation. Urea 80-84 carbamoyl-phosphate synthetase 1 Mus musculus 13-43 24703693-5 2014 We validated carbamoyl phosphate synthase 1 (CPS1), the rate-limiting enzyme in urea cycle, as a glutarylated protein and demonstrated that CPS1 is targeted by SIRT5 for deglutarylation. Urea 80-84 carbamoyl-phosphate synthetase 1 Mus musculus 45-49 24703693-5 2014 We validated carbamoyl phosphate synthase 1 (CPS1), the rate-limiting enzyme in urea cycle, as a glutarylated protein and demonstrated that CPS1 is targeted by SIRT5 for deglutarylation. Urea 80-84 carbamoyl-phosphate synthetase 1 Mus musculus 140-144 20097174-4 2010 We identified carbamoyl phosphate synthetase 1 (CPS1), a key enzyme of the urea cycle that catalyzes condensation of ammonia with bicarbonate to form carbamoyl phosphate, as a target of SIRT5 by two-dimensional electrophoresis comparing mitochondrial proteins in livers of SIRT5 Tg and wild-type mice. Urea 75-79 carbamoyl-phosphate synthetase 1 Mus musculus 14-46 20097174-4 2010 We identified carbamoyl phosphate synthetase 1 (CPS1), a key enzyme of the urea cycle that catalyzes condensation of ammonia with bicarbonate to form carbamoyl phosphate, as a target of SIRT5 by two-dimensional electrophoresis comparing mitochondrial proteins in livers of SIRT5 Tg and wild-type mice. Urea 75-79 carbamoyl-phosphate synthetase 1 Mus musculus 48-52 19268669-4 2009 RESULTS: Under normal circumstances, the urea cycle enzyme carbamoylphosphate synthetase I (CPS I) is present in the periportal, intermediate, and the first few layers of the perivenous zone. Urea 41-45 carbamoyl-phosphate synthetase 1 Mus musculus 59-90 19268669-4 2009 RESULTS: Under normal circumstances, the urea cycle enzyme carbamoylphosphate synthetase I (CPS I) is present in the periportal, intermediate, and the first few layers of the perivenous zone. Urea 41-45 carbamoyl-phosphate synthetase 1 Mus musculus 92-97 19410549-3 2009 Here, we show that SIRT5 localizes in the mitochondrial matrix and interacts with carbamoyl phosphate synthetase 1 (CPS1), an enzyme, catalyzing the initial step of the urea cycle for ammonia detoxification and disposal. Urea 169-173 carbamoyl-phosphate synthetase 1 Mus musculus 82-114 19410549-3 2009 Here, we show that SIRT5 localizes in the mitochondrial matrix and interacts with carbamoyl phosphate synthetase 1 (CPS1), an enzyme, catalyzing the initial step of the urea cycle for ammonia detoxification and disposal. Urea 169-173 carbamoyl-phosphate synthetase 1 Mus musculus 116-120 9862865-0 1999 Mice deficient in the urea-cycle enzyme, carbamoyl phosphate synthetase I, die during the early neonatal period from hyperammonemia. Urea 22-26 carbamoyl-phosphate synthetase 1 Mus musculus 41-73 11997094-6 2002 Citrin, ASS, CPS and Ornt1 showed similar patterns of developmental changes in the liver and small intestine, where they play a role in urea and arginine synthesis. Urea 136-140 carbamoyl-phosphate synthetase 1 Mus musculus 13-16 11136545-3 2000 We have found that all of the urea cycle enzyme genes are suppressed and that N-acetylglutamate, an allosteric activator of the first step enzyme of the urea cycle, carbamoyl phosphate synthetase I (CPS), is not deficient in the liver of JVS mice. Urea 30-34 carbamoyl-phosphate synthetase 1 Mus musculus 199-202 11136545-3 2000 We have found that all of the urea cycle enzyme genes are suppressed and that N-acetylglutamate, an allosteric activator of the first step enzyme of the urea cycle, carbamoyl phosphate synthetase I (CPS), is not deficient in the liver of JVS mice. Urea 153-157 carbamoyl-phosphate synthetase 1 Mus musculus 165-197 11136545-3 2000 We have found that all of the urea cycle enzyme genes are suppressed and that N-acetylglutamate, an allosteric activator of the first step enzyme of the urea cycle, carbamoyl phosphate synthetase I (CPS), is not deficient in the liver of JVS mice. Urea 153-157 carbamoyl-phosphate synthetase 1 Mus musculus 199-202 9862865-3 1999 Carbamoyl phosphate synthetase I (CPSase I; EC 6.3.4.16) is located within the inner mitochondrial matrix and catalyzes the initial rate-limiting step of the urea cycle. Urea 158-162 carbamoyl-phosphate synthetase 1 Mus musculus 0-32 9862865-3 1999 Carbamoyl phosphate synthetase I (CPSase I; EC 6.3.4.16) is located within the inner mitochondrial matrix and catalyzes the initial rate-limiting step of the urea cycle. Urea 158-162 carbamoyl-phosphate synthetase 1 Mus musculus 34-42 8733888-6 1996 In SCAD-deficient mice we found depressed mRNA expression and enzyme activity for the urea cycle enzymes CPS and AS at 6 days of age, and found no apparent effects on expression of gluconeogenic enzymes PC or PEPCK. Urea 86-90 carbamoyl-phosphate synthetase 1 Mus musculus 105-108 32359471-1 2020 The urea cycle enzyme carbamoyl phosphate synthetase 1 (CPS1) catalyzes the initial step of the urea cycle; bi-allelic mutations typically present with hyperammonemia, vomiting, ataxia, lethargy progressing into coma, and death due to brain edema if ineffectively treated. Urea 4-8 carbamoyl-phosphate synthetase 1 Mus musculus 22-54 32359471-1 2020 The urea cycle enzyme carbamoyl phosphate synthetase 1 (CPS1) catalyzes the initial step of the urea cycle; bi-allelic mutations typically present with hyperammonemia, vomiting, ataxia, lethargy progressing into coma, and death due to brain edema if ineffectively treated. Urea 4-8 carbamoyl-phosphate synthetase 1 Mus musculus 56-60 32359471-1 2020 The urea cycle enzyme carbamoyl phosphate synthetase 1 (CPS1) catalyzes the initial step of the urea cycle; bi-allelic mutations typically present with hyperammonemia, vomiting, ataxia, lethargy progressing into coma, and death due to brain edema if ineffectively treated. Urea 96-100 carbamoyl-phosphate synthetase 1 Mus musculus 22-54 32359471-1 2020 The urea cycle enzyme carbamoyl phosphate synthetase 1 (CPS1) catalyzes the initial step of the urea cycle; bi-allelic mutations typically present with hyperammonemia, vomiting, ataxia, lethargy progressing into coma, and death due to brain edema if ineffectively treated. Urea 96-100 carbamoyl-phosphate synthetase 1 Mus musculus 56-60 32359471-8 2020 This study demonstrates the first proof-of-principle that sAAV-mediated therapy is a viable, potentially clinically translatable approach to CPS1 deficiency, a devastating urea cycle disorder. Urea 172-176 carbamoyl-phosphate synthetase 1 Mus musculus 141-145 31016736-10 2019 Of urea cycle-related genes, arginase 1 (Arg1), ornithine transcarbamylase (Otc), and carbamoyl-phosphate synthase 1 (Cps1) expressions were up-regulated in Rev-erbalpha-/- mice. Urea 3-7 carbamoyl-phosphate synthetase 1 Mus musculus 86-116 31016736-10 2019 Of urea cycle-related genes, arginase 1 (Arg1), ornithine transcarbamylase (Otc), and carbamoyl-phosphate synthase 1 (Cps1) expressions were up-regulated in Rev-erbalpha-/- mice. Urea 3-7 carbamoyl-phosphate synthetase 1 Mus musculus 118-122 30843237-2 2019 When compared with mice that are highly repopulated with CPS1-proficient human hepatocytes, mice that are repopulated with CPS1-deficient human hepatocytes exhibited characteristic symptoms of human CPS1 deficiency including an 80% reduction in CPS1 metabolic activity, delayed clearance of an ammonium chloride infusion, elevated glutamine and glutamate levels, and impaired metabolism of [15 N]ammonium chloride into urea, with no other obvious phenotypic differences. Urea 419-423 carbamoyl-phosphate synthetase 1 Mus musculus 123-127 35285892-0 2022 Regulation of the urea cycle by CPS1 O-GlcNAcylation in response to dietary restriction and aging. Urea 18-22 carbamoyl-phosphate synthetase 1 Mus musculus 32-36 35285892-8 2022 Our results identify CPS1 O-GlcNAcylation as a key nutrient-sensing regulatory step in the urea cycle during aging and dietary restriction, implying a role for mitochondrial O-GlcNAcylation in nutritional regulation of longevity. Urea 91-95 carbamoyl-phosphate synthetase 1 Mus musculus 21-25 35202247-7 2022 FMO3-deficient mice showed hepatic overexpression of carbamoylphosphate synthetase (CPS1), the rate-limiting gene of urea cycle, and increased hepatic urea levels, especially in mice of FVB (Friend leukemia virus B strain) background. Urea 117-121 carbamoyl-phosphate synthetase 1 Mus musculus 84-88