Search by BoMiProt ID - Bomi226

Primary Information

BoMiProt ID Bomi226
Protein Name Argininosuccinate lyase
Organism Bos taurus
Uniprot IDQ3SZJ0
Milk FractionExosome
Ref Sequence ID NP_001029600.1
Aminoacid Length 473
Molecular Weight 52743
FASTA Sequence Download
Gene Name ASL
Gene ID 512771
Protein Existence Status Reviewed: Experimental evidence at transcript level

Secondary Information

Presence in other biological fluids/tissue/cells bovine liver, bovine kidney, all human tissues, cultured human skin fibroblasts, found in greatest concentrations in the livers of urea-forming animals
Protein Function catalyzes argininosuccinate = arginine + fumarate; catalyzes the fourth reaction of the Krebs-Henseleit ornithine cycle; biosynthesis of arginine from ornithine or citrulline. Argininosuccinate lyase (ASL) catalyzes the fourth reaction in this cycle, resulting in the breakdown of argininosuccinic acid to arginine and fumarate.
Biochemical Properties specific activity of 10.3 nmol min'1 mg'1 in the forward, argininosuccinate cleaving, reaction and 8.0 nmol min^-1 mg^-1 in the reverse reaction; enzyme appears to be a tetramer composed of subunits of identical molecular weight; Km values were 0.20 mM for argininosuccinate, 5.3 mM for fumarate, and 3.0 mM for arginine; The enzyme exhibited normal Michaelis-Menten kinetics, and guanosine 5'-triphosphate (GTP) had no affect on the activity of the enzyme
Significance in milk dietary supply of arginine in milk
PTMs N-acetylation at Alanine,N6-Acetylation at Lys
Site(s) of PTM(s)

N-glycosylation, O-glycosylation,
Predicted Disorder Regions NA
DisProt Annotation
TM Helix Prediction No TM helices
Significance of PTMs Acetylation on Lys-288 is decreased on the addition of extra amino acids resulting in activation of enzyme activity.
Additional Comments ASLD is typically established with elevation of plasma citrulline together with elevated argininosuccinic acid in the plasma or urine. 
Bibliography 1. O’Brien, W. E. and Barr, R. H. (1981) ‘Argininosuccinate lyase: purification and characterization from human liver’, Biochemistry, 20(7), pp. 2056–2060. doi: 10.1021/bi00510a049. 2.Nagamani SC, Erez A, Lee B. Argininosuccinate lyase deficiency. Genet Med. 2012 May;14(5):501-7. doi: 10.1038/gim.2011.1. Epub 2012 Jan 5. PMID: 22241104; PMCID: PMC3709024.