Home  >  Products  >  ADSL, Recombinant, Human, aa1-484, His-tag (Adenylosuccinate Lyase, Adenylosuccinase, ASase, ASL, AMPS)

ADSL, Recombinant, Human, aa1-484, His-tag (Adenylosuccinate Lyase, Adenylosuccinase, ASase, ASL, AMPS)

Cat no: A0909-60E


Supplier: United States Biological
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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). Source: Recombinant corresponding to aa1-484 from human ADSL, fused to His-tag at N-terminal expressed in E.coli. AA Sequence: MRGSHHHHHH GMASMTGGQQ MGRDLYDDDD KDRWGSMAAG 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 Molecular Weight: ~59kD Storage and Stability: May be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
Catalogue number: A0909-60E
Size: 100ug
Form: Supplied as a liquid in 20mM Tris-HCl buffer, pH 8.0, 1mM DTT, 40% glycerol, 0.1M sodium chloride.
Purity: ~95% (SDS-PAGE)
References: 1. Marie S., et al. (2002) Am J Hum Genet. 71:14-21. 2. Kmoch S., et al. (2000) 3. Hum Mal Genet. 9:1501-1513.

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