ID:ARLY_HUMAN DESCRIPTION: RecName: Full=Argininosuccinate lyase; Short=ASAL; EC=4.3.2.1; AltName: Full=Arginosuccinase; CATALYTIC ACTIVITY: 2-(N(omega)-L-arginino)succinate = fumarate + L-arginine. ENZYME REGULATION: Enzyme activity is regulated by acetylation (By similarity). PATHWAY: Amino-acid biosynthesis; L-arginine biosynthesis; L- arginine from L-ornithine and carbamoyl phosphate: step 3/3. PATHWAY: Nitrogen metabolism; urea cycle; L-arginine and fumarate from (N(omega)-L-arginino)succinate: step 1/1. SUBUNIT: Homotetramer. PTM: Acetylation modifies enzyme activity in response to alterations of extracellular nutrient availability. Acetylation increased with trichostin A (TSA) or with nicotinamide (NAM). Glucose increases acetylation by about a factor of 3 with decreasing enzyme activity. Acetylation on Lys-288 is decreased on the addition of extra amino acids resulting in activation of enzyme activity. DISEASE: Defects in ASL are the cause of arginosuccinic aciduria (ARGINSA) [MIM:207900]. 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. SIMILARITY: Belongs to the lyase 1 family. Argininosuccinate lyase subfamily. SEQUENCE CAUTION: Sequence=AAA51786.1; Type=Frameshift; Positions=387, 452; WEB RESOURCE: Name=GeneReviews; URL="http://www.ncbi.nlm.nih.gov/sites/GeneTests/lab/gene/ASL";
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on P04424
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
US Server
Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
