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Deficiency of liver arginase (AI) is characterized clinically by hyperargininemia, progressive mental impairment, growth retardation, spasticity, and periodic episodes of hyperammonemia. The rarest of the inborn errors of urea cycle enzymes, it has been considered the least life-threatening, by virtue of the typical absence of catastrophic neonatal(More)
A method is described for quantification of specific proteins after electrophoresis and transfer to immobilization membranes for Western blots. This method is analogous to methods used to correct the amounts of specific transcripts detected on Northern blots. Ponceau S staining of proteins bound to immobilization membranes is efficient and accurate compared(More)
Previous studies in our laboratory and others have demonstrated in humans and other mammals two isozymes of arginase (AI and AII) that differ both electrophoretically and antigenically. AI, a cytosolic protein found predominantly in liver and red blood cells, is believed to be chiefly responsible for ureagenesis and is the one missing in hyperargininemic(More)