| Clone |
PAT16C10AT. |
| Applications |
The antibody has been tested by ELISA, Western blot analysis, Flow cytometry and ICC/IF to assure specificity and reactivity. Since application varies, however, each investigation should be titrated by the reagent to obtain optimal results. |
| Protein Background |
Adenylosuccinate lyase (ADSL) is an enzyme which converts adenylosuccinate to AMP and fumarate as part of the purine nucleotide cycle. ADSL is involved in both de novo synthesis of purines and formation of adenosine monophosphate from inosine monophosphate. ADSL catalyzes 2 reactions in AMP biosynthesis: the removal of a fumarate from succinylaminoimidazole carboxamide (SAICA) ribotide to yield aminoimidazole carboxamide ribotide (AICA) and removal of fumarate from adenylosuccinate to yield AMP. Defects in the ADSL are the cause of adenylosuccinase deficiency (ADSL deficiency). ADSL deficiency is an autosomal recessive disorder distinguished by the accumulation in the body fluids of succinylaminoimidazole-carboxamide riboside (SAICA-riboside) and succinyladenosine (S-Ado). Adenylosuccinase deficiency results in succinylpurinemic autism, psychomotor retardation, and in some cases, growth retardation associated with muscle wasting and epilepsy. |
| Synonyms |
Adenylosuccinate lyase, ASL, Adenylosuccinase, ASase, ADSL, AMPS. |
| Reagent Appearance |
Sterile filtered colorless solution. |
| Type |
Mouse Anti Human Monoclonal. |
| Immunogen |
Anti-human ADSL mAb, is derived from hybridization of mouse F0 myeloma cells with spleen cells from BALB/c mice immunized with a recombinant human ADSL protein 1-484 amino acids purified from E. coli. |
| Ig Subclass |
Mouse IgG1 heavy chain and k light chain. |
| Purification Method |
ADSL antibody was purified from mouse ascitic fluids by protein-A affinity chromatography. |
| Protein formulation |
1mg/ml containing PBS, pH-7.4, 10% Glycerol and 0.02% Sodium Azide. |
| Storage Procedures |
For periods up to 1 month store at 4°C, for longer periods of time, store at -20°C. Prevent freeze thaw cycles. |
| Stability / Shelf Life |
12 months at -20°C. 1 month at 4°C. |
