BS8154

antibody from Bioworld Technology, Inc
Targeting: ASL ASAL
Provider product page for BS8154
Western blot
Immunohistochemistry

Antibody data

Product number
BS8154 - Provider product page
Provider
Bioworld Technology, Inc
Product name
ASL polyclonal antibody
Antibody type
Polyclonal
Antigen
Recombinant full length Human ASL.
Description
ASL (argininosuccinate lyase), also known as ASAL or arginosuccinase, is a member of the lyase 1 family of proteins and is predominantly expressed in the liver. Localizing to the cytoplasm and existing as a homotetramer, ASL catalyzes the hydrolytic cleavage of argininosuccinic acid (ASA) to fumarate and arginine, an essential step of the urea cycle which is crucial for the detoxification of ammonia. This reaction is also involved in the biosynthesis of arginine. In addition, ASL shares high sequence homology with the avian and reptilian eye lens protein, d-crystallin. Mutations in the gene encoding ASL leads to an accumulation of ASA in body fluids and results in Arginosuc-cinic aciduria (ASAuria), an autosomal recessive disorder that is characterized by hyperammonemia, liver enlargement, convulsions, physical and mental retardation, episodic unconsciousness and dry and brittle hair showing trich-orrhexis nodosa (weak points or nodes in the hair shaft).
Reactivity
Human, Mouse
Host
Rabbit
Isotype
IgG
Vial size
100ul
Concentration
1 mg/ml
Storage
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
ASL protein structure - BS8154 shown in red.