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Adenylosuccinate lyase

adenylosuccinate lyase, A DL
Top mentioned proteins: ACID, CAN, HAD, Ado, SIMPLE
Papers on adenylosuccinate lyase
Untargeted metabolomic analysis of urine samples in the diagnosis of some inherited metabolic disorders.
New
Adam et al., Olomouc, Czech Republic. In Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub, Dec 2015
METHODS: A total of 14 control urine samples and 21 samples from infants with cystinuria, maple syrup urine disease, adenylosuccinate lyase deficiency and galactosemia were tested.
Colocalization and Sequential Enzyme Activity in Aqueous Biphasic Systems: Experiments and Modeling.
New
Keating et al., United States. In Biophys J, Dec 2015
Two proteins of the de novo purine biosynthesis pathway, ASL (adenylosuccinate lyase, Step 8) and ATIC (5-aminoimidazole-4-carboxamide ribonucleotide transformylase/inosine monophosphate cyclohydrolase, Steps 9 and 10), were studied in a polyethylene glycol/dextran aqueous two-phase system.
Adenylosuccinate Is an Insulin Secretagogue Derived from Glucose-Induced Purine Metabolism.
New
Newgard et al., Durham, United States. In Cell Rep, Nov 2015
Inhibition of ADSS or a more proximal enzyme in the S-AMP biosynthesis pathway, adenylosuccinate lyase, lowers S-AMP levels and impairs GSIS.
Dual Learning in an Emergency Medicine Clerkship Improves Student Performance.
New
Coates et al., Los Angeles, United States. In J Emerg Med, Oct 2015
CONCLUSION: A DL model combining clinical and enhanced didactic requirements for an EM clerkship led to greater knowledge gain than the standard curriculum.
The use of a video laryngoscope by emergency medicine residents is associated with a reduction in esophageal intubations in the emergency department.
New
Stolz et al., Tucson, United States. In Acad Emerg Med, Jun 2015
The incidence of EI was compared between intubation attempts with a DL and with a VL (GlideScope(®) or C-MAC(®) ).
Fumaric acid production by Torulopsis glabrata: engineering the urea cycle and the purine nucleotide cycle.
Liu et al., Wuxi, China. In Biotechnol Bioeng, 2015
Using the genome-scale metabolic model iNX804 of T. glabrata, four fumaric acid biosynthetic pathways, involving the four cytosolic enzymes, argininosuccinate lyase (ASL), adenylosuccinate lyase (ADSL), fumarylacetoacetase (FAA), and fumarase (FUM1), were found.
Screening for adenylosuccinate lyase deficiency using tandem mass spectrometry analysis of succinylpurines in neonatal dried blood spots.
Kmoch et al., Praha, Czech Republic. In Clin Biochem, 2015
We present a method for LC-MS/MS determination of succinyladenosine (SAdo) and succinylaminoimidazole carboxamide riboside (SAICAr), biomarkers for adenylosuccinate lyase deficiency (dADSL), in DBS.
Inborn errors of purine metabolism: clinical update and therapies.
Christodoulou et al., Perth, Australia. In J Inherit Metab Dis, 2014
At present, treatments for the clinically significant defects of the purine pathway are restricted: purine 5'-nucleotidase deficiency with uridine; familial juvenile hyperuricaemic nephropathy (FJHN), adenine phosphoribosyl transferase (APRT) deficiency, hypoxanthine phosphoribosyl transferase (HPRT) deficiency and phosphoribosyl-pyrophosphate synthetase superactivity (PRPS) with allopurinol; adenosine deaminase (ADA) and purine nucleoside phosphorylase (PNP) deficiencies have been treated by bone marrow transplantation (BMT), and ADA deficiency with enzyme replacement with polyethylene glycol (PEG)-ADA, or erythrocyte-encapsulated ADA; myeloadenylate deaminase (MADA) and adenylosuccinate lyase (ADSL) deficiencies have had trials of oral ribose; PRPS, HPRT and adenosine kinase (ADK) deficiencies with S-adenosylmethionine; and molybdenum cofactor deficiency of complementation group A (MOCODA) with cyclic pyranopterin monophosphate (cPMP).
Attenuated adenylosuccinate lyase deficiency: a report of one case and a review of the literature.
Review
Tylki-Szymańska et al., Warsaw, Poland. In Neuropediatrics, 2014
We present a 9-year follow-up of a patient with an attenuated (type II) adenylosuccinate lyase deficiency with no obvious signs of disease progression and degradation.
Thirteen years experience with selective screening for disorders in purine and pyrimidine metabolism.
Review
Ugarte et al., Madrid, Spain. In Nucleosides Nucleotides Nucleic Acids, 2013
In the last thirteen years, we have studied urine/plasma specimens from about 1600 patients and we have identified 35 patients: eight patients with adenylosuccinate lyase deficiency, eight patients with hypoxanthine-guanine phosphoribosyltransferase deficiency, one patient with purine nucleoside phosphorylase deficiency, ten patients with xanthine dehydrogenase deficiency, six patients with molybdenum cofactor deficiency and two patients with dihydropyrimidine dehydrogenase deficiency.
Aspartase/fumarase superfamily: a common catalytic strategy involving general base-catalyzed formation of a highly stabilized aci-carboxylate intermediate.
Review
Poelarends et al., Groningen, Netherlands. In Biochemistry, 2012
Members of the aspartase/fumarase superfamily share a common tertiary and quaternary fold, as well as a similar active site architecture; the superfamily includes aspartase, fumarase, argininosuccinate lyase, adenylosuccinate lyase, δ-crystallin, and 3-carboxy-cis,cis-muconate lactonizing enzyme (CMLE).
Magnetic resonance imaging of the brain in adenylosuccinate lyase deficiency: a report of seven cases and a review of the literature.
Review
Tylki-Szymanska et al., Warsaw, Poland. In Eur J Pediatr, 2012
Adenylosuccinate lyase (ADSL) deficiency is a rare autosomal recessive disorder of purine metabolism.
The PRPP synthetase spectrum: what does it demonstrate about nucleotide syndromes?
Review
de Brouwer et al., Brisbane, Australia. In Nucleosides Nucleotides Nucleic Acids, 2011
Theoretically, purine toxicity disorders would not be ameliorated by SAMe therapy, and this was confirmed for one adenylosuccinate lyase-deficient child.
A mutation in adenylosuccinate lyase associated with mental retardation and autistic features.
Impact
Dixon et al., Ann Arbor, United States. In Nat Genet, 1992
A point mutation in a purine nucleotide biosynthetic enzyme, adenylosuccinate lyase (ASL), segregates with the disorder.
An infantile autistic syndrome characterised by the presence of succinylpurines in body fluids.
Impact
Van den Berghe et al., In Lancet, 1984
The compounds are dephosphorylated derivatives of the intracellular metabolites adenylosuccinate and succinylaminoimidazole carboxamide ribotide, the two substrates of adenylosuccinase (adenylosuccinate lyase, EC 4.3.2.2).
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