The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
1/1000. Predicted molecular weight: 47 kDa.
1/10 - 1/50.
1/10 - 1/50. ab171870-Rabbit polyclonal IgG, is suitable for use as an isotype control with this antibody.
1/50 - 1/100. Perform heat mediated antigen retrieval with citrate buffer pH 6 before commencing with IHC staining protocol.
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.
Immunohistochemistry analysis of formalin-fixed paraffin-embedded Human colon carcinoma labeling ASS1with ab175607 at 1/10 followed by peroxidase conjugation of the secondary antibody and DAB staining.
Flow Cytometry - Anti-ASS1 antibody (ab175607)
Flow Cytometry analysis of Widr cells labeling ASS1 with ab175607 at 1/10 (bottom histogram) and a negative control cell (top histogram). FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.