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ASL Antibody (Center) Blocking Peptide

Synthetic peptide

     
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Product Information
Primary Accession P04424
Other Accession NP_001020114.1, NP_000039.2
Clone Names 90121070
Additional Information
Gene ID 435
Other Names Argininosuccinate lyase, ASAL, Arginosuccinase, ASL
Format Peptides are lyophilized in a solid powder format. Peptides can be reconstituted in solution using the appropriate buffer as needed.
StorageMaintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.
PrecautionsThis product is for research use only. Not for use in diagnostic or therapeutic procedures.
Protein Information
Name ASL
Function Catalyzes the reversible cleavage of L-argininosuccinate to fumarate and L-arginine, an intermediate step reaction in the urea cycle mostly providing for hepatic nitrogen detoxification into excretable urea as well as de novo L-arginine synthesis in nonhepatic tissues (PubMed:11747433, PubMed:11747432, PubMed:9045711, PubMed:22081021, PubMed:2263616). Essential regulator of intracellular and extracellular L-arginine pools. As part of citrulline-nitric oxide cycle, forms tissue-specific multiprotein complexes with argininosuccinate synthase ASS1, transport protein SLC7A1 and nitric oxide synthase NOS1, NOS2 or NOS3, allowing for cell-autonomous L- arginine synthesis while channeling extracellular L-arginine to nitric oxide synthesis pathway (PubMed:22081021).
Research Areas
Citations (0)
citation

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Background

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.

References

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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$ 277.78
Cat# BP10658c
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