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The protein encoded by OPLAH acts as a homodimer, using ATP hydrolysis to catalyze the conversion of 5-oxo-L-proline to L-glutamate. Additionally we are shipping 5-Oxoprolinase (ATP-Hydrolysing) Kits (10) and 5-Oxoprolinase (ATP-Hydrolysing) Proteins (3) and many more products for this protein.
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An autosomal recessive mode of inheritance for 5-oxoprolinase deficiency is further supported by the identification of a single mutation in all 9/14 parent sample sets investigated (except for the father of one patient whose result suggests homozygosity), and the absence of 5-oxoprolinuria in all tested heterozygotes
In this study, clinical, biochemical, and genetic aspects of five Chinese 5-oxoprolinuria patients with OPLAH or GSS gene mutations were investigated.
the cause of cellular ATP depletion in nephrotic cystinosis may be the futile cycle, formed between two ATP-dependant gamma-glutamyl cycle enzymes, gamma-glutamyl cysteine synthetase and 5-oxoprolinase
Oplah full body knock-out mice had higher 5-oxoproline levels coupled to increased oxidative stress, myocardial fibrosis and elevated filling pressures reminiscent of heart failure with preserved ejection fraction.
OPLAH depletion occurred as a result of cardiac injury, leading to elevated 5-oxoproline and oxidative stress, whereas OPLAH overexpression improved cardiac function after ischemic injury.
amino acid sequence determined
The protein encoded by this gene acts as a homodimer, using ATP hydrolysis to catalyze the conversion of 5-oxo-L-proline to L-glutamate. Defects in this gene are a cause of 5-oxoprolinase deficiency (OPLAHD).
, 5-oxoprolinase (ATP-hydrolysing)