The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 0.01 - 0.03 µg/ml. Detects a band of approximately 37 kDa (predicted molecular weight: 35 kDa).
Use a concentration of 3.75 µg/ml.
Nitrogen metabolism; urea cycle; L-ornithine and urea from L-arginine: step 1/1.
Involvement in disease
Defects in ARG1 are the cause of argininemia (ARGIN) [MIM:207800]; also known as hyperargininemia. Argininemia is a rare autosomal recessive disorder of the urea cycle. Arginine is elevated in the blood and cerebrospinal fluid, and periodic hyperammonemia occurs. Clinical manifestations include developmental delay, seizures, mental retardation, hypotonia, ataxia, progressive spastic quadriplegia.