5-oxoprolinase deficiency

Also Known As:

5-alpha-oxoprolinase deficiency; Oxoprolinuria Due To Oxoprolinase Deficiency; Oxoprolinuria due to 5-oxoprolinase deficiency

Networked: 7 relevant articles (1 outcomes, 0 trials/studies)

Relationship Network

Disease Context: Research Results

Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 933
- Inborn Genetic Diseases: 11939
  - Inborn Errors Metabolism: 1162
    - Inborn Errors Amino Acid Metabolism: 1
      - 5-oxoprolinase deficiency: 7
Nutritional and Metabolic Diseases: 15
- Metabolic Diseases: 10142
  - Inborn Errors Metabolism: 1162
    - Inborn Errors Amino Acid Metabolism: 1
      - 5-oxoprolinase deficiency: 7
Enzymes and Coenzymes: 1
- Enzymes: 152586
  - Hydrolases: 2166
    - Amidohydrolases: 43
      - Pyroglutamate Hydrolase: 20
        5-oxoprolinase deficiency: 7

Related Diseases

1.	Malnutrition (Nutritional Deficiencies)
2.	Sepsis (Septicemia)
3.	Glutathione synthetase deficiency

Experts

1.	van den Bersselaar, Luuk René: 1 article (01/2020)
2.	van den Brule, Judith Maria Dimphena: 1 article (01/2020)
3.	van der Hoeven, Johannes Gerardus: 1 article (01/2020)
4.	Ding, Yuan: 1 article (11/2015)
5.	Li, Xiyuan: 1 article (11/2015)
6.	Liu, Yupeng: 1 article (11/2015)
7.	Ma, Yanyan: 1 article (11/2015)
8.	Song, Jinqing: 1 article (11/2015)
9.	Wang, Qiao: 1 article (11/2015)
10.	Yang, Yanling: 1 article (11/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to 5-oxoprolinase deficiency:

1.	AntioxidantsIBA 11/01/2015 - "Antioxidant treatments were proved effective for the three patients with GSS deficiency but not for the two patients with 5-oxoprolinase deficiency."
2.	Pyrrolidonecarboxylic Acid (Pyroglutamic Acid)IBA 01/01/2013 - "A urine 5-oxoproline assay performed five days after admission was negative, suggesting no evidence of a 5-oxoprolinase deficiency. " 03/01/1999 - "In patients with defects in the synthesis, breakdown and metabolism of glutathione (GSH), like glutathione synthetase deficiency (GSD) and 5-oxoprolinase deficiency, urinary excretion of 5-oxoproline, an intermediate of the gamma-glutamyl cycle, is increased. "
3.	Glutathione (Reduced Glutathione)IBA 03/01/1999 - "In patients with defects in the synthesis, breakdown and metabolism of glutathione (GSH), like glutathione synthetase deficiency (GSD) and 5-oxoprolinase deficiency, urinary excretion of 5-oxoproline, an intermediate of the gamma-glutamyl cycle, is increased. " 06/01/2014 - "Acquired 5-oxoprolinase deficiency (penicillins) or the presence of other risk factors of glutathione depletion such as malnutrition or sepsis seems to be necessary for symptoms development. "
4.	Pyroglutamate Hydrolase (5 Oxoprolinase)IBA 08/01/2012 - "Here, we report the first molecularly characterized patients with 5-oxoprolinase deficiency due to a mutation in OPLAH (which encodes 5-oxoprolinase). "
5.	Penicillins (Penicillin)FDA Link 06/01/2014 - "Acquired 5-oxoprolinase deficiency (penicillins) or the presence of other risk factors of glutathione depletion such as malnutrition or sepsis seems to be necessary for symptoms development. "
6.	gamma-Glutamyltransferase (gamma-Glutamyl Transpeptidase)IBA 04/24/1998 - "gamma-Glutamyl transpeptidase deficiency is associated with GSH-emia and GSH-uria whereas 5-oxoprolinase deficiency is associated with 5-oxoprolinuria. "
7.	Floxacillin (Flucloxacillin)IBA 01/01/2020 - "Females and patients with sepsis, impaired kidney and/or liver function, malnutrition, advanced age, congenital 5-oxoprolinase deficiency and supratherapeutic acetaminophen and flucloxacillin dosage are associated with increased risk. "
8.	Acetaminophen (Paracetamol)FDA LinkGeneric 01/01/2020 - "Females and patients with sepsis, impaired kidney and/or liver function, malnutrition, advanced age, congenital 5-oxoprolinase deficiency and supratherapeutic acetaminophen and flucloxacillin dosage are associated with increased risk. "