ASL Antikörper (AA 301-400) (AbBy Fluor® 680)
Kurzübersicht für ASL Antikörper (AA 301-400) (AbBy Fluor® 680) (ABIN4996917)
Target
Alle ASL Antikörper anzeigenReaktivität
Wirt
Klonalität
Konjugat
Applikation
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Bindungsspezifität
- AA 301-400
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Homologie
- Human,Mouse,Rat,Dog,Pig,Horse
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Aufreinigung
- Purified by Protein A.
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Immunogen
- KLH conjugated synthetic peptide derived from human ASL/Argininosuccinate Lyase
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Isotyp
- IgG
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Applikationshinweise
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IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200 -
Beschränkungen
- Nur für Forschungszwecke einsetzbar
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Format
- Liquid
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Konzentration
- 1 μg/μL
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Buffer
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
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Konservierungsmittel
- ProClin
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Vorsichtsmaßnahmen
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
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Lagerung
- -20 °C
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Informationen zur Lagerung
- Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
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Haltbarkeit
- 12 months
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- ASL (Argininosuccinate Lyase (ASL))
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Andere Bezeichnung
- ASL/Argininosuccinate Lyase
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Hintergrund
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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).
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Gen-ID
- 435
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Pathways
- Response to Growth Hormone Stimulus
Target
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