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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. De plus, nous expédions 5-Oxoprolinase (ATP-Hydrolysing) Kits (8) et 5-Oxoprolinase (ATP-Hydrolysing) Protéines (3) et beaucoup plus de produits pour cette protéine.
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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 (Montrer GSS Anticorps) gene mutations were investigated.
the cause of cellular ATP depletion in nephrotic cystinosis may be the futile cycle (Montrer LRMP Anticorps), 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)