Adenylosuccinate Lyase (ADSL) Antibody
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Description
ADSL Antibody is a Rabbit Polyclonal antibody against ADSL. 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.
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Product specifications
| Category | Primary Antibodies |
| Immunogen Target | Adenylosuccinate Lyase (ADSL) |
| Host | Rabbit |
| Reactivity | Human, Mouse, Rat |
| Recommended Dilution | ELISA: 1 µg/ml, WB: 1/500 - 1/1000, IF/ICC: 1/20 - 1/100. Optimal dilutions/concentrations should be determined by the end user. |
| Clonality | Polyclonal |
| Conjugation | Unconjugated |
| Isotype | IgG |
| Purification | Purified by affinity chromatography. |
| Size 1 | 20 µl |
| Size 2 | 100 µl |
| Size 3 | 2 × 100 µl |
| Form | Liquid |
| Tested Applications | ELISA, WB, IF/ICC |
| Buffer | PBS, pH 7.3, containing 0.02% sodium azide, 50% glycerol. |
| Availability | Shipped within 5-10 working days. |
| Storage | Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
| Dry Ice | No |
| UniProt ID | P30566 |
| Gene ID | 158 |
| NCBI Accession | NP_000017.1 |
| Background | Antibody anti-ADSL |
| Status | RUO |
| Note | Concentration: > 0.2 mg/ml - |
Descripción
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Human ADSL (Adenylosuccinate lyase) ELISA Kit
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ADSL antibody
ADSL(adenylosuccinate lyase) is also named as AMPS, ASase, ASL and belongs to the lyase 1 family. It is an enzyme involved in 2 pathways of purine nucleotide metabolism and catalyzes cleavage of succinyl groups to yield fumarate(PMID:18524658). Defects in ADSL are the cause of adenylosuccinase deficiency(ADSL deficiency). In humans, mutations in ADSL lead to an inborn error of metabolism originally characterized by developmental delay, often with autistic features(PMID:20884265)..The ADSL enzymatic activity is reduced in lymphocytes and red blood cells of the patient with severe psychomotor retardation(PMID:9545543). It has 2 isoforms produced by alternative splicing.
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