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Phosphorylase ratio and susceptibility to malignant hyperthermia.

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The ratio of muscle phosphorylase a to total phosphorylase, expressed as a percent, was determined in vastus lateralis muscle of 26 patients to examine the efficacy of this parameter as a method for screening for susceptibility to malignant hyperthermia (MH). As standard screening, all patients also

[Effect of a centrogenic fever on phosphorylase and pyruvate kinase activity in rabbit tissues].

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Administration of pyrogenal into rabbits caused a decrease in the activity of phosphorylase A and pyruvate kinase in musculus gastrocnemius as well as pyruvate kinase -- in musculus soleus. The phosphorylase A activity was increased within 3 hrs after the drug administration but it was decreased

Phosphorylase activity and malignant hyperthermia.

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An unusual presentation of purine nucleoside phosphorylase deficiency mimicking systemic juvenile idiopathic arthritis complicated by macrophage activation syndrome.

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Systemic juvenile idiopathic arthritis (sJIA) is an inflammatory condition that presents with fever, rash and arthritis. At onset systemic features are predominant and the diagnosis may be a challenge. Secondary hemophagocytic lymphohistiocytosis (sHLH) forms may be associated with

Phase I dose-escalating trial of Escherichia coli purine nucleoside phosphorylase and fludarabine gene therapy for advanced solid tumors.

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BACKGROUND The use of Escherichia coli purine nucleoside phosphorylase (PNP) to activate fludarabine has demonstrated safety and antitumor activity during preclinical analysis and has been approved for clinical investigation. METHODS A first-in-human phase I clinical trial (NCT 01310179; IND 14271)

A case report of papilla Vater carcinoma showing positive expression of thymidine phosphorylase.

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Recently, we experienced an interesting case of papilla Vater carcinoma. The patient was a 61-year-old woman, who was referred to our department from a private hospital with a chief complaint of high fever. Magnetic resonance cholangiopancreatography and endoscopic retrograde

Purine nucleoside phosphorylase deficiency in a patient with spastic paraplegia and recurrent infections.

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Purine nucleoside phosphorylase deficiency is a rare autosomal recessive immunodeficiency disease. The characteristic features of the disease include severe T cell immune defects with recurrent infections, a failure to thrive, and progressive neurological findings. To date, 35 cases of purine

Phase I clinical and pharmacological studies of benzylacyclouridine, a uridine phosphorylase inhibitor.

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Benzylacyclouridine (BAU, IND 039655) is a potent and specific inhibitor of uridine phosphorylase (UrdPase; EC 2.4.2.3). This enzyme plays a major role in regulating uridine homeostasis and also catalyzes the conversion of fluoropyrimidine nucleosides to their respective bases. Inhibition of UrdPase

Polynucleotide phosphorylase (PNPase) is required for Salmonella enterica serovar Typhimurium colonization in swine.

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The pnp gene encodes polynucleotide phosphorylase, an exoribonuclease involved in RNA processing and degradation. A mutation in the pnp gene was previously identified by our group in a signature-tagged mutagenesis screen designed to search for Salmonella enterica serovar Typhimurium genes required

[Histological, histochemical, and ultrastructural findings in malignant hyperthermia (author's transl)].

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Malignant hyperthermia is a rare but severe complication of modern anesthesia, induced by halothane and succinylcholine. The syndrome is characterized by a rapid sustained and extreme rise in body temperature associated with muscular rigidity, tachycardia, tachypnoea and cyanosis. The lethality is

Purine nucleoside phosphorylase deficiency associated with a fatal lymphoproliferative disorder.

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A 2 1/2-year-old boy presented with an illness resembling glandular fever. He was found to have T-cell deficiency with lack of red and white cell purine nucleoside phosphorylase enzyme activity. A spastic tetraparesis was noted. The patient subsequently died of a malignant lymphoma of the

Pitfalls in the histochemical demonstration of alpha-glucan phosphorylase activity in glycogen-depleted skeletal muscle fibres.

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In the present communication, an investigation is described into the reliability of histochemical methods for the demonstration of alpha-glucan phosphorylase activity in glycogen-depleted skeletal muscle fibres. Human skeletal muscles with glycogen-depleted fibres from patients with diseases of the

High skeletal muscle adenylate cyclase in malignant hyperthermia.

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Malignant hyperthermia occurs in humans with several congenital myopathies, usually in response to general anesthesia. Commonly, individuals who develop this syndrome lack symptoms of muscle disease, and their muscle lacks specific pathological changes. A biochemical marker for this myopathy has not

Histochemical observations on muscle from normal and malignant hyperthermia-susceptible swine.

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A comparison was made of muscle from two locations in both the longissimus and the semitendinous muscles of normal and malignant hyperthermia-susceptible swine. Serial frozen sections were stained for alkali-stable adenosine triphosphatase (ATPase), phosphorylase, and the oxidative enzymes succinate

Antitumor effects of a dual-specific lentiviral vector carrying the Escherichia coli purine nucleoside phosphorylase gene.

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The Escherichia coli purine nucleoside phosphorylase/Fludarabine phosphate (ePNP/Fludara) suicide system has several drawbacks, such as side-effects and the low efficiency of ePNP expression. In this study, we evaluated the antitumor effects of the dual-specific 8HSEs-hTERTp-ePNP/Fludara suicide

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