Results 11 to 20 of about 2,442 (103)

Hypoaminoacidemia and Pyroglutamic Aciduria: Potential Biomarkers in Malnutrition-Related Hyperammonemia. [PDF]

JIMD Rep
ABSTRACT Hyperammonemia is a medical emergency, and the cause must be identified quickly in order to treat appropriately. Malnutrition is a known risk factor for hyperammonemia; however, there are limited reliable lab indicators used to identify malnutrition.
Crenshaw MM, D'Annibale OM, Martucci V, Gracie S, Kochhar A, Stansauk J, Larson A, Baker P, Peck C, Wood T, El-Gharbawy A, McCandless S, LoPiccolo MK.   +12 more
europepmc   +2 more sources

Unveiling the Potential of Lentilactobacillus hilgardii in Malolactic Fermentation: Comparative Genomics and Fermentation Dynamics. [PDF]

Microb Biotechnol
This study explores the metabolic potential of Lentilactobacillus hilgardii for malolactic fermentation in winemaking. Genomic and in silico analyses, combined with experimental validation, reveal its resilience at low temperatures and unique enzymatic traits. Comparative insights with Oenococcus oeni highlight L.
Mantegazza G, Mangieri N, Yazdi EV, Russo P, Mora D, Gargari G.   +5 more
europepmc   +2 more sources

Metformin Inhibits the Urea Cycle and Reduces Putrescine Generation in Colorectal Cancer Cell Lines

Molecules, 2021
The urea cycle (UC) removes the excess nitrogen and ammonia generated by nitrogen-containing compound composites or protein breakdown in the human body.
Tao Zhang, Ling Hu, Jia-Feng Tang, Hang Xu, Kuan Tian, Meng-Na Wu, Shi-Ying Huang, Yu-Mei Du, Peng Zhou, Rui-Jin Lu, Shuang He, Jia-Mei Xu, Jian-Jun Si, Jing Li, Di-Long Chen, Jian-Hua Ran   +15 more
doaj   +1 more source

Corticosteroid suppresses urea-cycle-related gene expressions in ornithine transcarbamylase deficiency

BMC Gastroenterology, 2022
Background Ornithine transcarbamylase deficiency (OTCD) is most common among urea cycle disorders (UCDs), defined by defects in enzymes associated with ureagenesis.
Koji Imoto, Masatake Tanaka, Takeshi Goya, Tomomi Aoyagi, Motoi Takahashi, Miho Kurokawa, Shigeki Tashiro, Masaki Kato, Motoyuki Kohjima, Yoshihiro Ogawa   +9 more
doaj   +1 more source

Molecular and cellular mechanisms underlying gyrate atrophy: Why is the retina primarily affected? [PDF]

Acta Ophthalmol
Abstract Gyrate atrophy of the choroid and retina (GACR; OMIM #258870) is a rare early‐onset autosomal recessive disorder, caused by bi‐allelic pathogenic variants in the gene coding for ornithine aminotransferase (OAT) resulting in hyperornithinaemia.
Buijs MJN, Balfoort BM, Brands MM, Ten Asbroek ALMA, Boon CJF, Diederen RMH, Timmer C, Wagenmakers MAEM, Waterham HR, Wanders RJA, Houtkooper RH, van Karnebeek CD, Bergen AA.   +12 more
europepmc   +2 more sources

HMG-CoA Synthase-2 Deficiency: Neonatal Hyperammonemic Coma and Abnormal Metabolic Screening Resembling Maple Syrup Urine Disease. [PDF]

JIMD Rep
ABSTRACT Mitochondrial HMG‐CoA synthase‐2 (HMGCS2) deficiency is characterized by hypoketotic hypoglycemia, metabolic acidosis, hepatomegaly, and encephalopathy with onset between 3 and 36 months of age. Approximately 50 cases were reported worldwide. We describe two patients with HMGCS2 deficiency.
Vaseenon H, Tim-Aroon T, Saengow VE, Sangcakul A, Wongkittichote P, Khongkraparn A, Wattanasirichaigoon D.   +6 more
europepmc   +2 more sources

Association between the blood concentrations of ammonia and carnitine/amino acid of schizophrenic patients treated with valproic acid

BioPsychoSocial Medicine, 2017
Background Administration of valproic acid (VPA) is complicated with approximately 0.9% of patients developing hyperammonemia, but the pathogenesis of this adverse effect remains to be clarified.
Masazumi Ando, Hideaki Amayasu, Takahiro Itai, Hisahiro Yoshida   +3 more
doaj   +1 more source

The loss of the urea cycle and ornithine metabolism in different insect orders: An omics approach. [PDF]

Insect Mol Biol
Among urea cycle enzymes, only the nitric oxide synthase gene is universally present across insect genomes. All Hemiptera species lack the enzymes needed to convert citrulline to arginine, and some also lack the pathway from arginine to ornithine. Putrescine and spermidine synthesis is conserved in all insects, but aphids lack the capability to produce
Martins JCS, Assunção Romão HA, Canettieri CK, Cercilian AC, Oliveira PRO, Ferreira C, Terra WR, de Oliveira Dias R.   +7 more
europepmc   +2 more sources

N‐carbamoylglutamate‐responsive carbamoyl phosphate synthetase 1 (CPS1) deficiency: A patient with a novel CPS1 mutation and an experimental study on the mutation's effects

JIMD Reports, 2019
N‐carbamoyl‐l‐glutamate (NCG), the N‐acetyl‐l‐glutamate analogue used to treat N‐acetylglutamate synthase deficiency, has been proposed as potential therapy of carbamoyl phosphate synthetase 1 deficiency (CPS1D).
Sufin Yap, Nadine Gougeard, Anthony R. Hart, Belén Barcelona, Vicente Rubio   +4 more
doaj   +1 more source

Distinct Defence Mechanisms of Allelopathic Rice Against Quinclorac‐Susceptible and ‐Resistant Barnyardgrass: Involvement of Specific Metabolites and Rhizosheath Microbiota

Plant Biotechnology Journal, EarlyView.
ABSTRACT Allelopathic rice is increasingly recognised as a promising strategy for sustainable weed management. Resistance to the herbicide quinclorac is widespread in barnyardgrass, but it remains unclear whether allelopathic rice exerts the same defence against herbicide‐susceptible and ‐resistant barnyardgrass. We conducted integrated transcriptomic,
Shuyan Li, Qiling Yan, Jianhua Tong, Yongfeng Li, Lianyang Bai, Qiong Peng   +5 more
wiley   +1 more source