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Disease on EC 3.5.2.9 - 5-oxoprolinase (ATP-hydrolysing)

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DISEASE
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5-oxoprolinase (atp-hydrolysing) deficiency
5-Oxoprolinase deficiency associated with severe psychomotor developmental delay, failure to thrive, microcephaly and microcytic anaemia.
5-Oxoprolinase deficiency: report of the first human OPLAH mutation.
5-Oxoprolinuria associated with 5-oxoprolinase deficiency; further evidence that this is a benign disorder.
5-oxoprolinuria due to hereditary 5-oxoprolinase deficiency in two brothers--a new inborn error of the gamma-glutamyl cycle.
5-Oxoprolinuria in Heterozygous Patients for 5-Oxoprolinase (OPLAH) Missense Changes.
5-Oxoprolinuria in patients with and without defects in the gamma-glutamyl cycle.
A newborn infant with generalized glutathione synthetase deficiency.
Acetaminophen Use Concomitant with Long-Lasting Flucloxacillin Therapy: A Dangerous Combination.
Clinical, biochemical, and molecular characterization of patients with glutathione synthetase deficiency.
Coma, metabolic acidosis, and methemoglobinemia in a patient with acetaminophen toxicity.
Five Chinese patients with 5-oxoprolinuria due to glutathione synthetase and 5-oxoprolinase deficiencies.
Growth failure, encephalopathy, and endocrine dysfunctions in two siblings, one with 5-oxoprolinase deficiency.
Inborn errors in the metabolism of glutathione.
New insights into the genetics of 5-oxoprolinase deficiency and further evidence that it is a benign biochemical condition.
Patients with genetic defects in the gamma-glutamyl cycle.
Pyroglutamic acid-induced metabolic acidosis: a case report.
Unravelling 5-oxoprolinuria (pyroglutamic aciduria) due to bi-allelic OPLAH mutations: 20 new mutations in 14 families.
[5-Oxoprolinase deficiency]
Acidosis
Transient 5-oxoprolinuria (pyroglutamic aciduria) with systemic acidosis in an adult receiving antibiotic therapy.
Anemia
5-Oxoprolinase deficiency associated with severe psychomotor developmental delay, failure to thrive, microcephaly and microcytic anaemia.
Anemia, Hemolytic
Patients with genetic defects in the gamma-glutamyl cycle.
Unravelling 5-oxoprolinuria (pyroglutamic aciduria) due to bi-allelic OPLAH mutations: 20 new mutations in 14 families.
Brain Diseases
Growth failure, encephalopathy, and endocrine dysfunctions in two siblings, one with 5-oxoprolinase deficiency.
Breast Neoplasms
Glutamine affects glutathione recycling enzymes in a DMBA-induced breast cancer model.
Drug-Related Side Effects and Adverse Reactions
Potential for selective modulation of glutathione in cancer chemotherapy.
gamma-glutamyltransferase deficiency
Patients with genetic defects in the gamma-glutamyl cycle.
glutathione synthase deficiency
5-Oxoprolinuria in patients with and without defects in the gamma-glutamyl cycle.
A newborn infant with generalized glutathione synthetase deficiency.
Clinical, biochemical, and molecular characterization of patients with glutathione synthetase deficiency.
Patients with genetic defects in the gamma-glutamyl cycle.
Pyroglutamic aciduria (5-oxoprolinuria) without glutathione synthetase deficiency and with decreased pyroglutamate hydrolase activity.
Unravelling 5-oxoprolinuria (pyroglutamic aciduria) due to bi-allelic OPLAH mutations: 20 new mutations in 14 families.
Heart Failure
Heart failure and the glutathione cycle: an integrated view.
OPLAH ablation leads to accumulation of 5-oxoproline, oxidative stress, fibrosis, and elevated fillings pressures: a murine model for heart failure with a preserved ejection fraction.
Malnutrition
Acetaminophen Use Concomitant with Long-Lasting Flucloxacillin Therapy: A Dangerous Combination.
Pyroglutamic acid-induced metabolic acidosis: a case report.
membrane alanyl aminopeptidase deficiency
Inborn errors in the metabolism of glutathione.
Microcephaly
5-Oxoprolinase deficiency associated with severe psychomotor developmental delay, failure to thrive, microcephaly and microcytic anaemia.
Neoplasms
Activity and distribution of the cysteine prodrug activating enzyme, 5-oxo-L-prolinase, in human normal and tumor tissues.
Characterization of 5-oxo-L-prolinase in normal and tumor tissues of humans and rats: a potential new target for biochemical modulation of glutathione.
Glutamine affects glutathione recycling enzymes in a DMBA-induced breast cancer model.
Increased expression of the MGMT repair protein mediated by cysteine prodrugs and chemopreventative natural products in human lymphocytes and tumor cell lines.
Modulation of glutathione by a cysteine pro-drug enhances in vivo tumor response.
Novel methylated DNA markers accurately discriminate Lynch syndrome associated colorectal neoplasia.
Selective glutathione repletion with oral oxothiazolidine carboxylate (OTZ) in the radiated tumor-bearing rat.
Sensitization effect of L-2-oxothiazolidine-4-carboxylate on tumor cells to melphalan and the role of 5-oxo-L-prolinase in glutathione modulation in tumor cells.
Sepsis
Acetaminophen Use Concomitant with Long-Lasting Flucloxacillin Therapy: A Dangerous Combination.
Pyroglutamic acid-induced metabolic acidosis: a case report.