Rabbit anti-Human Adenylosuccinate Lyase Polyclonal Antibody | anti-ADSL antibody

Anti-Adenylosuccinate Lyase Antibody, Rabbit Polyclonal

Cat. Num. AAA8105226

• Gene Names: ADSL; ASL; AMPS; ASASE
• Reactivity: Human
• Applications: Western Blot, ELISA
• Purity: Protein A & Antigen Affinity
• Synonyms: Adenylosuccinate Lyase; Polyclonal Antibody; Anti-Adenylosuccinate Lyase Antibody; Rabbit Polyclonal; ADSL/Adenylosuccinate Lyase Antibody; Rabbit PAb; Antigen Affinity Purified; adenylosuccinate lyase; Anti-AMPS Antibody; Anti-ASASE Antibody; Anti-ASL Antibody; anti-ADSL antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: Rabbit IgG
• Specificity: Human ADSL/Adenylosuccinate Lyase
• Purity/Purification: Protein A & Antigen Affinity
• Form/Format: Liquid; 0.2um filtered solution in PBS

• Applicable Applications for anti-ADSL antibody: Western Blot (WB), ELISA (EIA)
• Application Notes: WB: 1:500-1:2000; ELISA: 1:5000-1:10,000
• Immunogen: Recombinant Human ADSL/Adenylosuccinate Lyase protein
• Conjugation: Unconjugated
• Preparation: Produced in rabbits immunized with purified, recombinant Human ADSL/Adenylosuccinate Lyase (rh ADSL/Adenylosuccinate Lyase). ADSL/Adenylosuccinate Lyase specific IgG was purified by Human ADSL/Adenylosuccinate Lyase affinity chromatography.
• Preparation and Storage: This antibody can be stored at 2-8 degree C for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20 degree C to -80 degree C. Preservative-Free. Avoid repeated freeze-thaw cycles.

Western Blot (WB)

(Anti-ADSL rabbit polyclonal antibody at 1:500 dilutionLane A: HepG2 Whole Cell LysateLysates/proteins at 30 ug per lane.SecondaryGoat Anti-Rabbit IgG H&L (Dylight800) at 1/10000 dilution.Developed using the Odyssey technique. Performed under reducing conditions.Predicted band size:55 kDaObserved band size:55 kDa)

Related Product Information for anti-ADSL antibody

Adenylosuccinate lyase, also known as adenylosuccinase, ADSL or 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 _-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 different mutations to the enzyme.

References

Nassogne M, et al. (2000) Adenylosuccinase deficiency: an unusual cause of early-onset epilepsy associated with acquired microcephaly. Brain and development. 22 (6): 383-6.Sivendran S, et al. (2004) Two novel mutant human adenylosuccinate lyases (ASLs) associated with autism and characterization of the equivalent mutant Bacillus subtilis ASL. J Biol Chem. 279 (51): 53789-97.Lee TT, et al. (1999) His68 and His141 are critical contributors to the intersubunit catalytic site of adenylosuccinate lyase of Bacillus subtilis. Biochemistry. 38 (1): 22-32.

NCBI and Uniprot Product Information

• NCBI GI #: 4557269
• NCBI GeneID: 158
• NCBI Accession #: NP_000017.1
• NCBI GenBank Nucleotide #: NM_000026.2
• UniProt Accession #: P30566; O75495; Q5TI34; B0QY76
• Molecular Weight: 48,328 Da
• NCBI Official Full Name: adenylosuccinate lyase isoform a
• NCBI Official Synonym Full Names: adenylosuccinate lyase
• NCBI Official Symbol: ADSL
• NCBI Official Synonym Symbols: ASL; AMPS; ASASE
• NCBI Protein Information: adenylosuccinate lyase; adenylosuccinase
• UniProt Protein Name: Adenylosuccinate lyase
• Protein Family: Adenylosuccinate lyase
• UniProt Gene Name: ADSL
• UniProt Synonym Gene Names: AMPS; ASL; ASase
• UniProt Entry Name: PUR8_HUMAN

NCBI Description

Adenylsuccinate lyase is involved in both de novo synthesis of purines and formation of adenosine monophosphate from inosine monophosphate. It catalyzes two reactions in AMP biosynthesis: the removal of a fumarate from succinylaminoimidazole carboxamide (SAICA) ribotide to give aminoimidazole carboxamide ribotide (AICA) and removal of fumarate from adenylosuccinate to give AMP. Adenylosuccinase deficiency results in succinylpurinemic autism, psychomotor retardation, and , in some cases, growth retardation associated with muscle wasting and epilepsy. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]

Uniprot Description

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: EC 4.3.2.2; Nucleotide Metabolism - purine; 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: AMP biosynthetic process; purine ribonucleoside monophosphate biosynthetic process; metabolic process; nucleobase, nucleoside and nucleotide metabolic process; purine nucleotide biosynthetic process; protein tetramerization; purine base metabolic process; 'de novo' IMP biosynthetic process

Disease: Adenylosuccinase Deficiency