Recombinant human Adenylosuccinate lyase/ADSL protein

Adenylosuccinate lyase, also known as ADSL, is an enzyme that converts adenylosuccinate to AMP and fumarate as part of the purine nucleotide cycle. 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) . Recombinant human ADSL protein, fused to His-tag at N-terminus, was expressed in E. coli and purified by using conventional chromatography techniques.

Product Specifications

Product Name Alternative

Adenylosuccinate lyase, AMPS, ASASE, ASL

Expression System

E.coli

Antigen Species

Human

Tag

His-Tag

Applications

SDS-PAGE

Concentration

1 mg/mL (determined by Bradford assay)

Purity

> 95% by SDS-PAGE

Molecular Weight

59 kDa (520aa)

Additionnal Information

ADSL, Adenylosuccinate lyase, AMPS, ASASE, ASL, ATGP1275-10 µg, ATGP1275-20 µg, ATGP1275-50 µg, ATGP1275-100 µg, ATGP1275-250 µg, ATGP1275-500 µg, ATGP1275-1 mg, ATGP1275-10, ATGP1275-20, ATGP1275-50, ATGP1275-100, ATGP1275-250, ATGP1275-500, ATGP1275-1

References & Citations

Marie S., et al. (2002) Am J Hum Genet. 71:14-21.; ; Kmoch S., et al. (2000) Hum Mal Genet. 9:1501-1513.

Storage Conditions

Can be stored at 2°C to 8°C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.

Formulation

Liquid in. 20 mM Tris-HCl buffer (pH 8.0) containing 1mM DTT, 40% glycerol, 0.1M NaCl

Scientific Category

Metabolism

NCBI Accession Number

NP_000017

Uniprot Accession Number

P30566

Species

Human

AA Sequence

MAAG GDHGSPDSYR SPLASRYASP EMCFVFSDRY KFRTWRQLWL WLAEAEQTLG LPITDEQIQE MKSNLENIDF KMAAEEEKRL RHDVMAHVHT FGHCCPKAAG IIHLGATSCY VGDNTDLIIL RNALDLLLPK LARVISRLAD FAKERASLPT LGFTHFQPAQ LTTVGKRCCL WIQDLCMDLQ NLKRVRDDLR FRGVKGTTGT QASFLQLFEG DDHKVEQLDK MVTEKAGFKR AFIITGQTYT RKVDIEVLSV LASLGASVHK ICTDIRLLAN LKEMEEPFEK QQIGSSAMPY KRNPMRSERC CSLARHLMTL VMDPLQTASV QWFERTLDDS ANRRICLAEA FLTADTILNT LQNISEGLVV YPKVIERRIR QELPFMATEN IIMAMVKAGG SRQDCHEKIR VLSQQAASVV KQEGGDNDLI ERIQVDAYFS PIHSQLDHLL DPSSFTGRAS QQVQRFLEEE VYPLLKPYES VMKVKAELCL