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|Antigen||Argininosuccinate Lyase (ASL) Antibodies|
|Reactivity||Human, Mouse (Murine), Rat (Rattus) Alternatives|
Immunofluorescence (Paraffin-embedded Sections) (IF (p))
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|Purification||Purified by Protein A.|
|Immunogen||KLH conjugated synthetic peptide derived from human ASL/Argininosuccinate Lyase|
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|Alternative Name||Asl/Argininosuccinate Lyase (ASL Antibody Abstract)|
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).
Synonyms: Argininosuccinase, Argininosuccinate lyase, Arginosuccinase, ARLY_HUMAN, ASAL, ASL, EC 22.214.171.124.
|Pathways||Response to Growth Hormone Stimulus|
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|Application Notes||IF(IHC-P) 1:50-200|
|Restrictions||For Research Use only|
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|Buffer||Aqueous buffered solution containing 1 % BSA, 50 % glycerol and 0.09 % sodium azide.|
|Precaution of Use||This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.|
|Storage Comment||Store at 4°C|
|Expiry Date||12 months|