ASL anticorps (AA 301-400) (Cy5)

N° du produit ABIN1704717

Cet anticorps anti-ASL est un anticorps Lapin Polyclonal détectant ASL dans WB, IF (cc) et IF (p). Adapté pour Humain.

Aperçu rapide pour ASL anticorps (AA 301-400) (Cy5) (ABIN1704717)

Antigène: ASL (Argininosuccinate Lyase (ASL))

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp ASL est conjugé à/à la Cy5

Application: Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Détail du produit anti-ASL anticorps (Cy5)

Épitope: AA 301-400

Homologie: Human,Mouse,Rat,Dog,Pig,Horse

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human ASL/Argininosuccinate Lyase

Isotype: IgG

Information d'application

Indications d'application: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Agent conservateur: ProClin

Précaution d'utilisation: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Stock: -20 °C

Stockage commentaire: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Date de péremption: 12 months

Détails sur ASL

Antigène: ASL (Argininosuccinate Lyase (ASL))

Autre désignation: ASL/Argininosuccinate Lyase

Sujet:

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).

ID gène: 435

Pathways: Response to Growth Hormone Stimulus