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ASL/Argininosuccinate Lyase Antibody, Cy5 Conjugated

Applications

  • WB
  • IF(IHC-P)
  • IF(IHC-F)
  • IF(ICC)

Predicted Reactivity

  • Human
  • Mouse
  • Rat
  • Dog
  • Pig
  • Horse
Overview
Catalog # bs-12515R-Cy5
Product Name ASL/Argininosuccinate Lyase Antibody, Cy5 Conjugated
Applications WB, IF(IHC-P), IF(IHC-F), IF(ICC)
Predicted Reactivity Human, Mouse, Rat, Dog, Pig, Horse
Specifications
Conjugation Cy5
Host Rabbit
Source KLH conjugated synthetic peptide derived from human ASL/Argininosuccinate Lyase
Immunogen Range 301-400/464
Clonality Polyclonal
Isotype IgG
Concentration 1ug/ul
Purification Purified by Protein A.
Storage Buffer Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage Condition Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
Target
Gene ID 435
Subcellular location Cytoplasm, Extracellular matrix
Synonyms Argininosuccinase; Argininosuccinate lyase; Arginosuccinase; ARLY_HUMAN; ASAL; ASL; EC 4.3.2.1.
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).
Application Dilution
WB 1:300-5000
IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200