DATASHEET
Host:
Rabbit
Target Protein:
ASL/Argininosuccinate Lyase
Immunogen Range:
301-400/464
Clonality:
Polyclonal
Isotype:
IgG
Entrez Gene:
435
Source:
KLH conjugated synthetic peptide derived from human ASL/Argininosuccinate Lyase
Purification:
Purified by Protein A.
Storage Buffer:
Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Storage:
Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
Background:
ASL 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).
Conjugation:
PE
Excitation/ Emission:
496,564nm/578nm
Size:
100ul
Concentration:
1ug/ul
Applications:
WB(1:300-5000)
52
Predicted Cross Reactive Species:
Human
Mouse
Rat
Dog
Pig
Horse
For research use only. Not intended for diagnostic or therapeutic use.