Title : Understanding pyrroline-5-carboxylate synthetase deficiency: clinical, molecular, functional, and expression studies, structure-based analysis, and novel therapy with arginine.

Pub. Date : 2012 Sep

PMID : 22170564






3 Functional Relationships(s)
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Sentence
Compound Name
Protein Name
Organism
1 Delta(1)-Pyrroline-5-carboxylate synthetase (P5CS) catalyzes the first two steps of ornithine/proline biosynthesis. Proline aldehyde dehydrogenase 18 family member A1 Homo sapiens
2 Delta(1)-Pyrroline-5-carboxylate synthetase (P5CS) catalyzes the first two steps of ornithine/proline biosynthesis. Proline aldehyde dehydrogenase 18 family member A1 Homo sapiens
3 Only one family exhibited metabolic changes consistent with P5CS deficiency (low proline/ornithine/citrulline/arginine; fasting hyperammonemia). Proline aldehyde dehydrogenase 18 family member A1 Homo sapiens