product summary
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company name :
MyBioSource
product type :
antibody
product name :
ASL Antibody
catalog :
MBS9412831
quantity :
0.1 mL
price :
225 USD
clonality :
polyclonal
host :
rabbit
conjugate :
nonconjugated
reactivity :
human
application :
immunohistochemistry
more info or order :
MyBioSource product webpage
product information
catalog number :
MBS9412831
products type :
Antibody
products full name :
ASL Antibody
products short name :
ASL
products name syn :
ASAL
other names :
argininosuccinate lyase isoform 1; Argininosuccinate lyase; argininosuccinate lyase; argininosuccinate lyase; Arginosuccinase
products gene name :
ASL
other gene names :
ASL; ASL; ASAL; ASAL
uniprot entry name :
ARLY_HUMAN
clonality :
Polyclonal
host :
Rabbit
reactivity :
Human
sequence length :
464
specificity :
The antibody detects endogenous levels of total ASL protein.
purity :
Antigen affinity purification.
form :
Rabbit IgG in pH7.3 PBS, 0.05% NaN3, 50% Glycerol.
concentration :
1.4 mg/ml
storage stability :
Store at -20 degree C
tested application :
Immunohistochemistry (IHC)
app notes :
Immunohistochemistry: 1:50-1:200
other info1 :
Immunogen Type: Recombinant Protein. Immunogen Description: Fusion protein corresponding to residues near the C terminal of human argininosuccinate lyase
other info2 :
Target Name: ASL
products categories :
Total protein Ab
products description :
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.
ncbi gi num :
31541964
ncbi acc num :
NP_000039.2
ncbi gb acc num :
NM_000048.3
uniprot acc num :
P04424
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 Biosynthesis Pathway (82943); Arginine Biosynthesis Pathway (306); Biosynthesis Of Amino Acids Pathway (790012); Biosynthesis Of Amino Acids Pathway (795174); Biosynthesis Of Antibiotics Pathway (1144997); L-citrulline-nitric Oxide Cycle Pathway (703105); Metabolic Pathways (132956)
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: EC 4.3.2.1; Amino Acid Metabolism - arginine and proline; Amino Acid Metabolism - alanine, aspartate and glutamate; Lyase. 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
size1 :
0.1 mL
price1 :
225 USD
more info or order :
MyBioSource product webpage
company information
MyBioSource
P.O. Box 153308
San Diego, CA 92195-3308
sales@mybiosource.com
https://www.mybiosource.com
1-888-627-0165
headquarters: USA
MyBioSource, LLC was orginally founded in Vancouver by three enthusiastic scientists who are passionate about providing the world with the best reagents available. Together, they form a company with a big vision known as MyBioSource. MyBioSource is now located in San Diego, California, USA.

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