ASL Antikörper (AA 301-400) (Biotin)

Produktnummer ABIN1701094

Dieses Kaninchen Polyklonal-Antikörper erkennt spezifisch ASL in WB, ELISA, IHC (p) und IHC (fro). Er zeigt eine Reaktivität gegenüber Human.

Kurzübersicht für ASL Antikörper (AA 301-400) (Biotin) (ABIN1701094)

Target: ASL (Argininosuccinate Lyase (ASL))

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser ASL Antikörper ist konjugiert mit Biotin

Applikation: Western Blotting (WB), ELISA, Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))

Produktdetails anti-ASL Antikörper (Biotin)

Bindungsspezifität: AA 301-400

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

Aufreinigung: Purified by Protein A.

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

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: WB 1:300-5000
IHC-P 1:200-400
IHC-F 1:100-500

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 μg/μL

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

Konservierungsmittel: ProClin

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

Lagerung: -20 °C

Informationen zur Lagerung: Store at -20°C for 12 months.

Haltbarkeit: 12 months

Details zu ASL

Target: ASL (Argininosuccinate Lyase (ASL))

Andere Bezeichnung: ASL/Argininosuccinate Lyase

Hintergrund:

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).

Gen-ID: 435

Pathways: Response to Growth Hormone Stimulus