PMID-sentid Pub_year Sent_text comp_official_name comp_offsetprotein_name organism prot_offset 26829900-9 2016 ALDH18A1 encodes Delta(1)-pyrroline-5-carboxylate synthase, which is related to the biosynthesis of ornithine, citrulline, arginine, and proline. Ornithine 100-109 aldehyde dehydrogenase 18 family member A1 Homo sapiens 0-8 26829900-9 2016 ALDH18A1 encodes Delta(1)-pyrroline-5-carboxylate synthase, which is related to the biosynthesis of ornithine, citrulline, arginine, and proline. Ornithine 100-109 aldehyde dehydrogenase 18 family member A1 Homo sapiens 17-58 18401542-1 2008 Mammalian Delta(1)-pyrroline-5-carboxylate synthase (P5CS) is a bifunctional ATP- and NAD(P)H-dependent mitochondrial enzyme that catalyzes the coupled phosphorylation and reduction-conversion of L: -glutamate to P5C, a pivotal step in the biosynthesis of L: -proline, L: -ornithine and L: -arginine. Ornithine 269-282 aldehyde dehydrogenase 18 family member A1 Homo sapiens 10-51 26026163-3 2015 ALDH18A1 encodes delta-1-pyrroline-5-carboxylate synthase (P5CS), an enzyme that catalyses the first and common step of proline and ornithine biosynthesis from glutamate. Ornithine 132-141 aldehyde dehydrogenase 18 family member A1 Homo sapiens 0-8 26026163-3 2015 ALDH18A1 encodes delta-1-pyrroline-5-carboxylate synthase (P5CS), an enzyme that catalyses the first and common step of proline and ornithine biosynthesis from glutamate. Ornithine 132-141 aldehyde dehydrogenase 18 family member A1 Homo sapiens 17-57 26026163-3 2015 ALDH18A1 encodes delta-1-pyrroline-5-carboxylate synthase (P5CS), an enzyme that catalyses the first and common step of proline and ornithine biosynthesis from glutamate. Ornithine 132-141 aldehyde dehydrogenase 18 family member A1 Homo sapiens 59-63 26026163-6 2015 Low levels of plasma ornithine, citrulline, arginine and proline in four individuals from two families suggested P5CS deficiency. Ornithine 21-30 aldehyde dehydrogenase 18 family member A1 Homo sapiens 113-117 22170564-1 2012 Delta(1)-Pyrroline-5-carboxylate synthetase (P5CS) catalyzes the first two steps of ornithine/proline biosynthesis. Ornithine 84-93 aldehyde dehydrogenase 18 family member A1 Homo sapiens 0-43 22170564-1 2012 Delta(1)-Pyrroline-5-carboxylate synthetase (P5CS) catalyzes the first two steps of ornithine/proline biosynthesis. Ornithine 84-93 aldehyde dehydrogenase 18 family member A1 Homo sapiens 45-49 22170564-3 2012 Only one family exhibited metabolic changes consistent with P5CS deficiency (low proline/ornithine/citrulline/arginine; fasting hyperammonemia). Ornithine 89-98 aldehyde dehydrogenase 18 family member A1 Homo sapiens 60-64 18401542-1 2008 Mammalian Delta(1)-pyrroline-5-carboxylate synthase (P5CS) is a bifunctional ATP- and NAD(P)H-dependent mitochondrial enzyme that catalyzes the coupled phosphorylation and reduction-conversion of L: -glutamate to P5C, a pivotal step in the biosynthesis of L: -proline, L: -ornithine and L: -arginine. Ornithine 269-282 aldehyde dehydrogenase 18 family member A1 Homo sapiens 53-57 18401542-3 2008 The short form (P5CS.short) is highly expressed in the gut and is inhibited by ornithine. Ornithine 79-88 aldehyde dehydrogenase 18 family member A1 Homo sapiens 16-20 10037775-10 1999 We found that both confer P5CS activity and that the short isoform is inhibited by L-ornithine with a Ki of approximately 0.25 mM. Ornithine 83-94 aldehyde dehydrogenase 18 family member A1 Homo sapiens 26-30 18478038-8 2008 In an in vivo assay of flux through this metabolic pathway using dermal fibroblasts obtained from an affected individual, proline and ornithine biosynthetic activity of P5CS was not affected by the H784Y substitution. Ornithine 134-143 aldehyde dehydrogenase 18 family member A1 Homo sapiens 169-173 15517380-1 2005 UNLABELLED: Delta1-pyrroline-5-carboxylate synthase (P5CS) catalyses the reduction of glutamate to Delta1-pyrroline-5-carboxylate, a critical step in the biosynthesis of proline, ornithine and arginine. Ornithine 179-188 aldehyde dehydrogenase 18 family member A1 Homo sapiens 18-51 15517380-1 2005 UNLABELLED: Delta1-pyrroline-5-carboxylate synthase (P5CS) catalyses the reduction of glutamate to Delta1-pyrroline-5-carboxylate, a critical step in the biosynthesis of proline, ornithine and arginine. Ornithine 179-188 aldehyde dehydrogenase 18 family member A1 Homo sapiens 53-57 15517380-8 2005 Furthermore, we show the results of ornithine loading tests and indirect enzyme studies corroborating the biological significance of the defect in P5CS in vivo. Ornithine 36-45 aldehyde dehydrogenase 18 family member A1 Homo sapiens 147-151 11092761-0 2000 Hyperammonemia with reduced ornithine, citrulline, arginine and proline: a new inborn error caused by a mutation in the gene encoding delta(1)-pyrroline-5-carboxylate synthase. Ornithine 28-37 aldehyde dehydrogenase 18 family member A1 Homo sapiens 134-175 11092761-1 2000 delta(1)-pyrroline-5-carboxylate synthase (P5CS), a bifunctional ATP- and NADPH-dependent mitochondrial enzyme, catalyzes the reduction of glutamate to delta(1)-pyrroline-5-carboxylate, a critical step in the biosynthesis of proline, ornithine and arginine. Ornithine 234-243 aldehyde dehydrogenase 18 family member A1 Homo sapiens 0-41 11092761-1 2000 delta(1)-pyrroline-5-carboxylate synthase (P5CS), a bifunctional ATP- and NADPH-dependent mitochondrial enzyme, catalyzes the reduction of glutamate to delta(1)-pyrroline-5-carboxylate, a critical step in the biosynthesis of proline, ornithine and arginine. Ornithine 234-243 aldehyde dehydrogenase 18 family member A1 Homo sapiens 43-47 10037775-2 1999 Delta1-Pyrroline-5-carboxylate synthase (P5CS; EC not assigned), a mitochondrial inner membrane, ATP- and NADPH-dependent, bifunctional enzyme, catalyzes the reduction of glutamate to Delta1-pyrroline-5-carboxylate, a critical step in the de novo biosynthesis of proline and ornithine. Ornithine 275-284 aldehyde dehydrogenase 18 family member A1 Homo sapiens 0-39 10037775-2 1999 Delta1-Pyrroline-5-carboxylate synthase (P5CS; EC not assigned), a mitochondrial inner membrane, ATP- and NADPH-dependent, bifunctional enzyme, catalyzes the reduction of glutamate to Delta1-pyrroline-5-carboxylate, a critical step in the de novo biosynthesis of proline and ornithine. Ornithine 275-284 aldehyde dehydrogenase 18 family member A1 Homo sapiens 41-45 10037775-12 1999 Thus, the two amino acid insert in the long isoform abolishes feedback inhibition of P5CS activity by L-ornithine. Ornithine 102-113 aldehyde dehydrogenase 18 family member A1 Homo sapiens 85-89 6115302-2 1981 Using a radioisotopic assay, we have studied the regulation by ornithine of delta 1-pyrroline-5-carboxylate synthase, the enzyme that catalyzes the first step of proline biosynthesis from glutamic acid. Ornithine 63-72 aldehyde dehydrogenase 18 family member A1 Homo sapiens 76-116 8761662-5 1996 The phenotypic features for deficiency of P5CS include joint hyperlaxity, skin hyperelasticity, cataract and mental retardation with hyperammonemia and low plasma levels of proline, citrulline and ornithine. Ornithine 197-206 aldehyde dehydrogenase 18 family member A1 Homo sapiens 42-46 35286254-1 2022 The bifunctional enzyme Delta1-pyrroline-5-carboxylate synthase (P5CS) is vital to the synthesis of proline and ornithine, playing an essential role in human health and agriculture. Ornithine 112-121 aldehyde dehydrogenase 18 family member A1 Homo sapiens 24-63 35286254-1 2022 The bifunctional enzyme Delta1-pyrroline-5-carboxylate synthase (P5CS) is vital to the synthesis of proline and ornithine, playing an essential role in human health and agriculture. Ornithine 112-121 aldehyde dehydrogenase 18 family member A1 Homo sapiens 65-69 32798076-1 2021 BACKGROUND: The ALDH18A1 gene is located at 10q24.1 and encodes delta-1-pyrroline-5-carboxylate synthetase (P5CS), a mitochondrial bifunctional enzyme that catalyzes the first two steps in de novo biosynthesis of proline, ornithine, citrulline, and arginine. Ornithine 222-231 aldehyde dehydrogenase 18 family member A1 Homo sapiens 16-24 32798076-1 2021 BACKGROUND: The ALDH18A1 gene is located at 10q24.1 and encodes delta-1-pyrroline-5-carboxylate synthetase (P5CS), a mitochondrial bifunctional enzyme that catalyzes the first two steps in de novo biosynthesis of proline, ornithine, citrulline, and arginine. Ornithine 222-231 aldehyde dehydrogenase 18 family member A1 Homo sapiens 64-106 32798076-1 2021 BACKGROUND: The ALDH18A1 gene is located at 10q24.1 and encodes delta-1-pyrroline-5-carboxylate synthetase (P5CS), a mitochondrial bifunctional enzyme that catalyzes the first two steps in de novo biosynthesis of proline, ornithine, citrulline, and arginine. Ornithine 222-231 aldehyde dehydrogenase 18 family member A1 Homo sapiens 108-112 33573605-11 2021 Analysis of serum amino acid levels revealed that two SPG9A patients and two unaffected family members had low citrulline levels and one had a low level of ornithine. Ornithine 156-165 aldehyde dehydrogenase 18 family member A1 Homo sapiens 54-59 32770108-1 2021 Pyrroline-5-carboxylate synthase (P5CS) catalyzes the synthesis of pyrroline-5-carboxylate (P5C), a key precursor for the synthesis of proline and ornithine. Ornithine 147-156 aldehyde dehydrogenase 18 family member A1 Homo sapiens 0-32 32770108-1 2021 Pyrroline-5-carboxylate synthase (P5CS) catalyzes the synthesis of pyrroline-5-carboxylate (P5C), a key precursor for the synthesis of proline and ornithine. Ornithine 147-156 aldehyde dehydrogenase 18 family member A1 Homo sapiens 34-38