scholarly journals A Role for Cytosolic Fumarate Hydratase in Urea Cycle Metabolism and Renal Neoplasia

Cell Reports ◽  
2013 ◽  
Vol 3 (5) ◽  
pp. 1440-1448 ◽  
Author(s):  
Julie Adam ◽  
Ming Yang ◽  
Christina Bauerschmidt ◽  
Mitsuhiro Kitagawa ◽  
Linda O’Flaherty ◽  
...  
Keyword(s):  
Urea Cycle ◽  
Fumarate Hydratase ◽  
Renal Neoplasia

scholarly journals Enzymes Catalyzing the TCA- and Urea Cycle Influence the Matrix Composition of Biofilms Formed by Methicillin-Resistant Staphylococcus aureus USA300

2018 ◽  
Vol 6 (4) ◽  
pp. 113 ◽  
Author(s):  
Sarah De Backer ◽  
Julia Sabirova ◽  
Ines De Pauw ◽  
Henri De Greve ◽  
Jean-Pierre Hernalsteens ◽  
...  
Keyword(s):  
Staphylococcus Aureus ◽  
Urea Cycle ◽  
Citrate Synthase ◽  
Fumarate Hydratase ◽  
Tca Cycle ◽  
Matrix Composition ◽  
Methicillin Resistant ◽  
The Tca Cycle ◽  
The Matrix ◽  
Biofilm Matrix

In methicillin-sensitive Staphylococcus aureus (MSSA), the tricarboxylic acid (TCA) cycle is known to negatively regulate production of the major biofilm-matrix exopolysaccharide, PIA/PNAG. However, methicillin-resistant S. aureus (MRSA) produce a primarily proteinaceous biofilm matrix, and contribution of the TCA-cycle therein remains unclear. Utilizing USA300-JE2 Tn-mutants (NARSA) in genes encoding TCA- and urea cycle enzymes for transduction into a prolific biofilm-forming USA300 strain (UAS391-Erys), we studied the contribution of the TCA- and urea cycle and of proteins, eDNA and PIA/PNAG, to the matrix. Genes targeted in the urea cycle encoded argininosuccinate lyase and arginase (argH::Tn and rocF::Tn), and in the TCA-cycle encoded succinyl-CoA synthetase, succinate dehydrogenase, aconitase, isocitrate dehydrogenase, fumarate hydratase class II, and citrate synthase II (sucC::Tn, sdhA/B::Tn, acnA::Tn, icd::Tn, fumC::Tn and gltA::Tn). Biofilm formation was significantly decreased under no flow and flow conditions by argH::Tn, fumC::Tn, and sdhA/B::Tn (range OD492 0.374−0.667; integrated densities 2.065−4.875) compared to UAS391-EryS (OD492 0.814; integrated density 10.676) (p ≤ 0.008). Cellular and matrix stains, enzymatic treatment (Proteinase K, DNase I), and reverse-transcriptase PCR-based gene-expression analysis of fibronectin-binding proteins (fnbA/B) and the staphylococcal accessory regulator (sarA) on pre-formed UAS391-Erys and Tn-mutant biofilms showed: (i) < 1% PIA/PNAG in the proteinaceous/eDNA matrix; (ii) increased proteins under no flow and flow in the matrix of Tn mutant biofilms (on average 50 and 51 (±11)%) compared to UAS391-Erys (on average 22 and 25 (±4)%) (p < 0.001); and (iii) down- and up-regulation of fnbA/B and sarA, respectively, in Tn-mutants compared to UAS391-EryS (0.62-, 0.57-, and 2.23-fold on average). In conclusion, we show that the biofilm matrix of MRSA-USA300 and the corresponding Tn mutants is PIA/PNAG-independent and are mainly composed of proteins and eDNA. The primary impact of TCA-cycle inactivation was on the protein component of the biofilm matrix of MRSA-USA300.

Mutation analysis of urea cycle disorders

2016 ◽  
Vol 04 (01) ◽  
pp. 033-043
Author(s):  
Johannes Häberle ◽  
Véronique Rüfenacht
Keyword(s):  
Mutation Analysis ◽  
Urea Cycle ◽  
Urea Cycle Disorders ◽  
Cycle Disorders

The Preparation and Function Identification of a Novel Anti-Fumarate Hydratase Antibody

2013 ◽  
Vol 29 (1) ◽  
pp. 46
Author(s):  
Meihong FU ◽  
Liang LE ◽  
Zhong CHENG ◽  
Yong XIE ◽  
Hai GAO ◽  
...  
Keyword(s):  
Fumarate Hydratase ◽  
Function Identification ◽  
And Function

P.24 Urea cycle intermediates in short bowel patientsfed with oral diet

1998 ◽  
Vol 17 ◽  
pp. 35
Author(s):  
A.M. Pita ◽  
Y. Wakabayashi ◽  
M.A. Femandez-Bustos ◽  
N. Virgili ◽  
M. Farriol
Keyword(s):  
Urea Cycle ◽  
Short Bowel ◽  
Oral Diet

Perinatal undernourishment provokes long-lasting alterations of clusterin and fumarate hydratase expression in the rat nucleus accumbens

2021 ◽  
pp. 1-5
Author(s):  
Carmen Rodríguez-Rivera ◽  
Carmen González-Martín ◽  
Elisa Fernández-Millán ◽  
Carmen Álvarez ◽  
Fernando Escrivá ◽  
...  
Keyword(s):  
Nucleus Accumbens ◽  
Fumarate Hydratase ◽  
Rat Nucleus Accumbens

Retrospective evaluation of 85 patients with urea cycle disorders: one center experience, three new mutations

2020 ◽  
Vol 33 (6) ◽  
pp. 721-728
Author(s):  
Özlem Saritaş Nakip ◽  
Yılmaz Yıldız ◽  
Ayşegül Tokatlı
Keyword(s):  
Mortality Rate ◽  
Urea Cycle ◽  
Laboratory Data ◽  
Patient Characteristics ◽  
Hereditary Diseases ◽  
Urea Cycle Disorders ◽  
Carbamoyl Phosphate ◽  
Term Survival ◽  
Genetic Features ◽  
Cycle Disorders

AbstractObjectivesUrea cycle disorders (UCDs) are rare hereditary diseases. This study was conducted to help identify the characteristics of UCDs in Turkey.MethodsThe primary outcome was to determine patient characteristics. Investigating the relationships between the patient outcomes and ammonia levels were the secondary outcomes. Eighty five patients from 79 families, diagnosed with UCD at a single metabolic referral center between 1979 and 2017, were included. Clinical and laboratory data were retrieved retrospectively from hospital records.ResultsClassical citrullinemia was the most common type of UCD; citrin deficiency and carbamoyl phosphate synthase 1 deficiency (CPS1D) were the rarest. One thirty one hyperammonemic episodes were recorded. The peak ammonia levels were found to be significantly associated with polycythemia and hypocalcemia at presentation. The median peak ammonia values of the patients who died were higher than those of the survivors. The highest mortality rate was in the classical citrullinemia group. The mortality rate of the first hyperammonemic crisis was 28.6%, while it was 6.7% in subsequent episodes with an odds ratio of 4.28 (95% CI: 1.67–11.0) (p=0.001). Forty-four patients underwent genetic analysis and genetic variants were detected in 42 patients (95%). Three of the detected variants have not been previously reported.ConclusionsThis is the largest UCD series in Turkey and may serve as a guide to clinical, biochemical and genetic features of UCDs in our country. Prevention of hyperammonemia may be the most influential measure to improve long term survival.

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