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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) Antibodies (13) and 5-Oxoprolinase (ATP-Hydrolysing) Kits (8) 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 (show GSS Proteins) gene mutations were investigated.
the cause of cellular ATP depletion in nephrotic cystinosis may be the futile cycle (show LRMP Proteins), formed between two ATP-dependant gamma-glutamyl cycle enzymes, gamma-glutamyl cysteine synthetase and 5-oxoprolinase
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)