ADSL, Polyclonal Antibody

AMPS; Adenylosuccinate lyase; ASL; Adenylosuccinase; ASase

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

Recognizes endogenous levels of ADSL protein.

The antibody was purified by immunogen affinity chromatography.

Liquid in 0.42% Potassium phosphate, 0.87% Sodium chloride, pH 7.3, 30% glycerol, and 0.01% sodium azide.

Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C for one year. Avoid freeze/thaw cycles.

Small volumes of anti-ADSL antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Rabbit polyclonal antibody to ADSL

Western Blot (WB), Immunofluorescence (IF), Immunocytochemistry (ICC)

WB: 1/500 - 1/2000; IF/ICC: 1/10 - 1/100

Western blot analysis of ADSL expression in HepG2 (A), Jurkat (B), mouse heart (C), rat brain (D) whole cell lysates.

Immunofluorescent analysis of ADSL staining in Hela cells. Formalin-fixed cells were permeabilized with 0.1% Triton X-100 in TBS for 5-10 minutes and blocked with 3% BSA-PBS for 30 minutes at room temperature. Cells were probed with the primary antibody in 3% BSA-PBS and incubated overnight at 4 °C in a humidified chamber. Cells were washed with PBST and incubated with a DyLight 594-conjugated secondary antibody (red) in PBS at room temperature in the dark.

48,328 Da

adenylosuccinate lyase

adenylosuccinate lyase

Adenylosuccinate lyase

AMPS; ASase  [Similar Products]

PUR8_HUMAN

The protein encoded by this gene belongs to the lyase 1 family. It is an essential enzyme involved in purine metabolism, and catalyzes two non-sequential reactions in the de novo purine biosynthetic pathway: the conversion of succinylaminoimidazole carboxamide ribotide (SAICAR) to aminoimidazole carboxamide ribotide (AICAR) and the conversion of adenylosuccinate (S-AMP) to adenosine monophosphate (AMP). Mutations in this gene are associated with adenylosuccinase deficiency (ADSLD), a disorder marked with psychomotor retardation, epilepsy or autistic features. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Dec 2015]

ADSL: 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). Most children display marked psychomotor delay, often accompanied by epilepsy or autistic features, or both, although some patients may be less profoundly retarded. Occasionally, growth retardation and muscular wasting are also present. Belongs to the lyase 1 family. Adenylosuccinate lyase subfamily. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Nucleotide Metabolism - purine; EC 4.3.2.2; Lyase; Amino Acid Metabolism - alanine, aspartate and glutamate

Chromosomal Location of Human Ortholog: 22q13.2

Cellular Component: cytosol

Molecular Function: adenylosuccinate lyase activity

Biological Process: 'de novo' IMP biosynthetic process; AMP biosynthetic process; protein tetramerization; purine nucleotide biosynthetic process; purine ribonucleoside monophosphate biosynthetic process

Disease: Adenylosuccinase Deficiency

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