ELISA/assay

Human Adenylosuccinate Lyase (ADSL) ELISA Kit

MBS9311768

48-Strip-Wells

435 USD

ELISA Kit

Human Adenylosuccinate Lyase (ADSL) ELISA Kit

Adenylosuccinate Lyase (ADSL)

adenylosuccinate lyase; Adenylosuccinate lyase; adenylosuccinate lyase; adenylosuccinase; adenylosuccinate lyase; Adenylosuccinase

ADSL

ADSL; ADSL; ASL; AMPS; ASASE; AMPS; ASL; ASase

PUR8_HUMAN

Human

No significant cross-reactivity or interference between this analyte and analogues is observed.

Store all reagents at 2-8 degree C

Samples: Undiluted original Human body fluids, tissue homogenates, secretions or feces samples. This kit is NOT suitable for assaying non-biological sources of substances. Assay Type: Sandwich. Detection Range: 0.625 ng/ml - 20 ng/ml. Sensitivity: 0.1 ng/ml.

Intra-assay Precision: Intra-assay CV (%) is less than 15%. Inter-assay Precision: Inter-assay CV (%) is less than 15%. [CV(%) = SD/mean ×100].

Background/Introduction: This Quantitative Sandwich ELISA kit is only for in vitro research use only, not for drug, household, therapeutic or diagnostic applications! This kit is intended to be used for determination the level of ADSL (hereafter termed "analyte") in undiluted original Human body fluids, tissue homogenates, secretions or feces samples. This kit is NOT suitable for assaying non-biological sources of substances.

3211982

AAC21560.1

P30566

54,889 Da

Adenine Ribonucleotide Biosynthesis, IMP = ADP,ATP Pathway (618580); Adenine Ribonucleotide Biosynthesis, IMP = ADP,ATP Pathway (468242); Alanine, Aspartate And Glutamate Metabolism Pathway (101142); Alanine, Aspartate And Glutamate Metabolism Pathway (100063); Inosine Monophosphate Biosynthesis, PRPP + Glutamine = IMP Pathway (413392); Inosine Monophosphate Biosynthesis, PRPP + Glutamine = IMP Pathway (468241); Metabolism Pathway (477135); Metabolism Of Nucleotides Pathway (106263); Nucleotide Metabolism Pathway (198876); Purine Metabolism Pathway (82944)

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]

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

48-Strip-Wells

435 USD

96-Strip-Wells

600