ADSL / Adenylosuccinate Lyase抗

ADSL / Adenylosuccinate Lyase抗体产品信息

免疫原 :
Recombinant Human ADSL / Adenylosuccinate Lyase protein (Catalog#11287-H07E)
Antibody Type : Rabbit Polyclonal Antibody ( Antibody Purification Platform )
抗体宿主 :
Rabbit IgG
缓冲液 : 0.2 μm filtered solution in PBS, 5% trehalose may be added in some batches. Please read the hardcopy of COA or contact our customer service to confirm the formulation.
制备方法 :
Produced in rabbits immunized with purified, recombinant Human ADSL / Adenylosuccinate Lyase (rh ADSL / Adenylosuccinate Lyase; Catalog#11287-H07E; P30566-1; Met1-Leu484). ADSL / Adenylosuccinate Lyase specific IgG was purified by Human ADSL / Adenylosuccinate Lyase affinity chromatography.
ADSL / Adenylosuccinate Lyase抗体 Background
Adenylosuccinate lyase, also known as adenylosuccinase, ADSL and ASL, is an enzyme implicated in the reaction of adenylosuccinat converting to AMP and fumarate as part of the purine nucleotide cycle. The two substates of adenylosuccinate lyase (ADSL) are dephosphorylated derivatives of SAICA ribotide (SAICAR) and adenylosuccinate (S-AMP), which catalyzes an important reaction in the de novo pathway of purine biosynthesis. ADSL catalyzes two distinct reactions in the synthesis of purine nucleotides, both of which involve the β-elimination of fumarate to produce either aminoimidazole carboxamide ribotide from SAICAR or AMP from S-AMP. The Adenylosuccinate lyase deficiency is a rare autosomal recessive metabolic disorder characterized by the present of SAICA riboside and succinyladenosine (S-Ado). ADSL defect in different patients is often caused by defferent mutations to the enzyme. More than 30 different mutations have been found worldwide. The mutations resulting in gene defects are spread throughout the enzyme, with the majority located far from the active site.
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