catalog number :
MBS144777
products type :
Recombinant Protein
products full name :
Recombinant Human Argininosuccinate Lyase
products short name :
[Argininosuccinate Lyase]
products name syn :
[ASL Human; Argininosuccinate Lyase Human Recombinant; Argininosuccinate lyase; ASAL; Arginosuccinase; ASL]
other names :
[argininosuccinate lyase isoform 1; Argininosuccinate lyase; argininosuccinate lyase; argininosuccinase; arginosuccinase; argininosuccinate lyase; Arginosuccinase]
products gene name :
[ASL]
other gene names :
[ASL; ASL; ASAL; ASAL]
uniprot entry name :
ARLY_HUMAN
sequence :
MGSSHHHHHH SSGLVPRGSH MASESGKLWG GRFVGAVDPI MEKFNASIAY DRHLWEVDVQ GSKAYSRGLE KAGLLTKAEM DQILHGLDKV AEEWAQGTFK LNSNDEDIHT ANERRLKELI GATAGKLHTG RSRNDQVVTD LRLWMRQTCS TLSGLLWELI RTMVDRAEAE RDVLFPGYTH LQRAQPIRWS HWILSHAVAL TRDSERLLEV RKRINVLPLG SGAIAGNPLG VDRELLRAEL NFGAITLNSM DATSERDFVA EFLFWASLCM THLSRMAEDL ILYCTKEFSF VQLSDAYSTG SSLMPQKKNP DSLELIRSKA GRVFGRCAGL LMTLKGLPST YNKDLQEDKE AVFEVSDTMS AVLQVATGVI STLQIHQENM GQALSPDMLA TDLAYYLVRK GMPFRQAHEA SGKAVFMAET KGVALNQLSL QELQTISPLF SGDVICVWDY GHSVEQYGAL GGTARSSVDW QIRQVRALLQ AQQA.
purity :
Greater than 95.0% as determined by SDS-PAGE.
form :
The ASL solution (0.5mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 2mM DTT, 10% glycerol and 100mM NaCl. Sterile Filtered colorless solution.
storage stability :
Store at 4 degree C if entire vial will be used within 2-4 weeks. Store, frozen at -20 degree C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA). Avoid multiple freeze-thaw cycles.
products categories :
ENZYMES; Enzymes; Lyase
products description :
Description: ASL Human Recombinant produced in E Coli is a single, non-glycosylated polypeptide chain containing 484 amino acids (1-464) and having a molecular mass of 53.8kDa.ASL is fused to a 20 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques. Introduction: Argininosuccinate lyase (ASL) is a member of the lyase 1 family. ASL is an enzyme which catalyzes the reversible breakdown of Argininosuccinate (ASA) yielding the amino acids arginine and fumarate. ASL which is located in the liver cytosol is the 4th enzyme of the urea cycle and involved in the biosynthesis of arginine in all species and the production of urea in ureotelic species. While Argininosuccinate synthetase (ASS) catalyzes the formation of argininosuccinate from citrulline and aspartate, ASL breaks down the newly formed argininosuccinate into L-arginine and fumarate. L-arginine continues within the urea cycle to form urea and orinthine, whereas fumarate can enter the citric acid cycle. ASL gene Mutations result in the autosomal recessive disorder argininosuccinic aciduria, or argininosuccinic acid lyase deficiency.
ncbi acc num :
NP_000039.2
ncbi gb acc num :
NM_000048.3
ncbi mol weight :
48,733 Da
ncbi pathways :
Alanine And Aspartate Metabolism Pathway (198783); Alanine, Aspartate And Glutamate Metabolism Pathway (101142); Alanine, Aspartate And Glutamate Metabolism Pathway (100063); Arginine And Proline Metabolism Pathway (82957); Arginine And Proline Metabolism Pathway (323); Biosynthesis Of Amino Acids Pathway (790012); Biosynthesis Of Amino Acids Pathway (795174); Metabolic Pathways (132956); Metabolism Pathway (477135); Metabolism Of Amino Acids And Derivatives Pathway (106169)
ncbi summary :
This gene encodes a member of the lyase 1 family. The encoded protein forms a cytosolic homotetramer and primarily catalyzes the reversible hydrolytic cleavage of argininosuccinate into arginine and fumarate, an essential step in the liver in detoxifying ammonia via the urea cycle. Mutations in this gene result in the autosomal recessive disorder argininosuccinic aciduria, or argininosuccinic acid lyase deficiency. A nontranscribed pseudogene is also located on the long arm of chromosome 22. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Jul 2008]
uniprot summary :
ASL: Defects in ASL are the cause of arginosuccinic aciduria (ARGINSA). An autosomal recessive disorder of the urea cycle. The disease is characterized by mental and physical retardation, liver enlargement, skin lesions, dry and brittle hair showing trichorrhexis nodosa microscopically and fluorescing red, convulsions, and episodic unconsciousness. Belongs to the lyase 1 family. Argininosuccinate lyase subfamily. Protein type: Lyase; EC 4.3.2.1; Amino Acid Metabolism - arginine and proline; Amino Acid Metabolism - alanine, aspartate and glutamate. Chromosomal Location of Human Ortholog: 7q11.21. Cellular Component: cytoplasm; cytosol. Molecular Function: argininosuccinate lyase activity. Biological Process: arginine catabolic process; arginine biosynthetic process via ornithine; internal protein amino acid acetylation; urea cycle. Disease: Argininosuccinic Aciduria