Human Adenylosuccinate Lyase/ADSL antibody
Adenylosuccinate lyase, also known as ADSL, is an enzyme that converts adenylosuccinate to AMP and fumarate as part of the purine nucleotide cycle. Defects in ADSL are the cause of adenylosuccinase deficiency (ADSL deficiency) . ADSL deficiency is an autosomal recessive disorder characterized by the accumulation in the body fluids of succinylaminoimidazole-carboxamide riboside (SAICA-riboside) and succinyladenosine (S-Ado) .
Product Specifications
Product Name Alternative
Adenylosuccinate lyase, AMPS, ASASE, ASL
Host
Mouse
Antigen Species
Human
Reactivity
Human
Immunogen
Recombinant human ADSL (1-484aa) purified from E. coli
Clonality
Monoclonal
Isotype
IgG1 κ
Clone
AT16C10
Conjugation
Unconjugated
Applications
ELISA, WB, ICC/IF, FACS
Purification Method
By protein-A affinity chromatography
Concentration
1 mg/mL (determined by BCA assay)
Additionnal Information
References & Citations
Marie S., et al. (2002) Am J Hum Genet. 71: 14-21. ; ; Kmoch S., et al. (2000) Hum Mal Genet. 9: 1501-1513.;
Storage Conditions
Can be stored at 2°C to 8°C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.
Formulation
Liquid in. Phosphate-Buffered Saline (pH 7.4) with 0.02% Sodium Azide, 10% glycerol
Applications Notes
Scientific Category
Metabolism
NCBI Accession Number
NP_000017
Uniprot Accession Number
P30566
WB Description
FACS Description
IF Description
Frequently Asked Questions
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