Catalogue number
ANT-623
Synonyms
Adenylosuccinate lyase, ASL, Adenylosuccinase, ASase,
ADSL, AMPS.
Type
Mouse Anti Human Monoclonal.
Introduction
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.
Clone
PAT16C10AT.
Physical Appearance
Sterile filtered colorless solution.
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.
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.
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.
Safety Data Sheet
Usage
ProSpec's products are furnished for LABORATORY RESEARCH USE ONLY. The product may not be used as drugs, agricultural or pesticidal products, food additives or household chemicals.
