|Other Accession||NP_001020114.1, NP_000039.2|
|Other Names||Argininosuccinate lyase, ASAL, Arginosuccinase, ASL|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
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Provided below are standard protocols that you may find useful for product applications.
ASL encodes a member of the lyase 1 family. Theencoded protein forms a cytosolic homotetramer and primarilycatalyzes the reversible hydrolytic cleavage of argininosuccinateinto arginine and fumarate, an essential step in the liver indetoxifying ammonia via the urea cycle. Mutations in this generesult in the autosomal recessive disorder argininosuccinicaciduria, or argininosuccinic acid lyase deficiency. Anontranscribed pseudogene is also located on the long arm ofchromosome 22. Alternatively spliced transcript variants encodingdifferent isoforms have been described.
Hozyasz, K.K., et al. Arch. Oral Biol. (2010) In press :Trevisson, E., et al. J. Biol. Chem. 284(42):28926-28934(2009)Trevisson, E., et al. Hum. Mutat. 28(7):694-702(2007)Tanaka, T., et al. Tohoku J. Exp. Med. 198(2):119-124(2002)Kleijer, W.J., et al. J. Inherit. Metab. Dis. 25(5):399-410(2002)
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