Anticorps Recombinant de lapin anti-ADSL

ADSL Recombinant Antibody for WB, Indirect ELISA

Hôte / Isotype

Lapin / IgG

Réactivité testée

Humain, rat, souris

Applications

WB, Indirect ELISA

Conjugaison

Non conjugué

CloneNo.

250216H4

N° de cat : 86131-2-PBS

Synonymes

AMPS, ASASE, ASL, EC:4.3.2.2

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Galerie de données de validation

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  WB analysis using 86131-2-RR (same clone as 86131-2-PBS)

  Various lysates were subjected to SDS PAGE followed by western blot with 86131-2-RR (ADSL antibody) at dilution of 1:5000 incubated at room temperature for 1.5 hours. This data was developed using the same antibody clone with 86131-2-PBS in a different storage buffer formulation.

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  Affinity and Kinetic Characterization of 86131-2-RR

  Biolayer interferometry (BLl) kinetic assays of 86131-2-RR against Human ADSL were performed. The affinity constant is 2.59 nM.

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Informations sur le produit

86131-2-PBS cible ADSL dans les applications de WB, Indirect ELISA et montre une réactivité avec des échantillons Humain, rat, souris

• Réactivité: Humain, rat, souris
• Hôte / Isotype: Lapin / IgG
• Clonalité: Recombinant
• Type: Anticorps
• Immunogène: ADSL Protéine recombinante Ag7332
• Nom complet: adenylosuccinate lyase
• Masse moléculaire calculée: 55 kDa
• Poids moléculaire observé: 55 kDa
• Numéro d’acquisition GenBank: BC000253
• Symbole du gène: ADSL
• Identification du gène (NCBI): 158
• Conjugaison: Non conjugué
• Forme: Liquide
• Méthode de purification: Purification par protéine A
• Tampon de stockage: PBS only
• Conditions de stockage: Store at -80°C. 20ul contiennent 0,1% de BSA.

Informations générales

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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