The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
1/500 - 1/2000. Detects a band of approximately 47 kDa (predicted molecular weight: 47 kDa).
1/200 - 1/400.
ab170190 - Mouse monoclonal IgG1, is suitable for use as an isotype control with this antibody.
1/200 - 1/1000.
Amino-acid biosynthesis; L-arginine biosynthesis; L-arginine from L-ornithine and carbamoyl phosphate: step 2/3. Nitrogen metabolism; urea cycle; (N(omega)-L-arginino)succinate from L-aspartate and L-citrulline: step 1/1.
Involvement in disease
Defects in ASS1 are the cause of citrullinemia type 1 (CTLN1) [MIM:215700]. Citrullinemia belongs to the urea cycle disorders. It is an autosomal recessive disease characterized primarily by elevated serum and urine citrulline levels. Ammonia intoxication is another manifestation. CTLN1 usually manifests in the first few days of life. Affected infants appear normal at birth, but as ammonia builds up in the body they present symptoms such as lethargy, poor feeding, vomiting, seizures and loss of consciousness. Less commonly, a milder CTLN1 form can develop later in childhood or adulthood.
Belongs to the argininosuccinate synthase family. Type 1 subfamily.
Western blot - Anti-ASS1 antibody [2B10] (ab124465)
Lane 1: Wild type HAP1 whole cell lysate (20 µg) Lane 2: ASS1 knockout HAP1 whole cell lysate (20 µg) Lane 3: HeLa whole cell lysate (20 µg) Lane 4: Jurkat whole cell lysate (20 µg)
Lanes 1 - 4: Merged signal (red and green). Green - ab124465 observed at 47 kDa. Red - loading control, ab181602, observed at 37 kDa.
ab124465 was shown to recognize ASS1 when ASS1 knockout samples were used, along with additional cross-reactive bands. Wild-type and ASS1 knockout samples were subjected to SDS-PAGE. ab124465 and ab181602 (Rabbit anti-GAPDH loading control) were incubated overnight at 4°C at a 1/500 dilution and 1/10000 dilution respectively. Blots were developed with Goat anti-Mouse IgG H&L (IRDye® 800CW) preabsorbed ab216772 and Goat anti-Rabbit IgG H&L (IRDye® 680RD) preabsorbed ab216777 secondary antibodies at 1/10000 dilution for 1 hour at room temperature before imaging.
Ohshima K et al. Argininosuccinate Synthase 1-Deficiency Enhances the Cell Sensitivity to Arginine through Decreased DEPTOR Expression in Endometrial Cancer. Sci Rep7:45504 (2017).
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