Arginase 1

• Description:

  Arginase-1 (ARG1) deficiency is a rare autosomal recessive disorder that affects the liver-based urea cycle, leading to impaired ureagenesis. This genetic disorder is caused by 40+ mutations found fairly uniformly spread throughout the ARG1 gene, resulting in partial or complete loss of enzyme function, which catalyzes the hydrolysis of arginine to ornithine and urea. ARG1-deficient patients exhibit hyperargininemia with spastic paraparesis, progressive neurological and intellectual impairment, persistent growth retardation, and infrequent episodes of hyperammonemia, a clinical pattern that differs strikingly from other urea cycle disorders.

• Synonyms:

  Liver-type arginase, Type I arginase, ARG1

• NCBI Gene ID:

  383

• UniProt:

  P05089

• Accession Number:

  NP_000036.2

• Accession Number mRNA:

  NM_000045.3

• Reactivity:

  Anti-Human

• Cross Reactivity:

  Human

• Target Antigen:

  Recombinant human Arginase 1

• Clone:

  (#41P23)

• Applications:

  WB, FC, IP

• Purification Method:

  Protein G chromatography

• Assay Protocol:

  Reconstitute the antibody with 500 µl sterile PBS and the final concentration is 200 µg/ml.

• Form:

  Lyophilized

• Buffer:

  PBS

• Reconstitution:

  PBS

• Storage Conditions:

  Lyophilized samples are stable for 2 years from date of receipt when stored at -70°C. Reconstituted antibody can be aliquoted and stored frozen at < -20 °C for at least for six months without detectable loss of activity.

• Host or Source:

  Mouse

• Isotype:

  IgG2