CLN5 Antikörper (AA 61-120) (AbBy Fluor® 488)

Produktnummer ABIN1392904

Produktdetails anti-CLN5 Antikörper (AbBy Fluor® 488)

Target: CLN5 (Ceroid-Lipofuscinosis, Neuronal 5 (CLN5))

Bindungsspezifität: AA 61-120

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser CLN5 Antikörper ist konjugiert mit AbBy Fluor® 488

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

Kreuzreaktivität: Human

Homologie: Mouse,Rat,Cow,Sheep,Pig,Horse,Rabbit

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human CLN5

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

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. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Haltbarkeit: 12 months

Details zu CLN5

Target: CLN5 (Ceroid-Lipofuscinosis, Neuronal 5 (CLN5))

Andere Bezeichnung: CLN5 (CLN5 Produkte)

Synonyme: NCL antikoerper, A730075N08Rik antikoerper, CLN5, intracellular trafficking protein antikoerper, ceroid-lipofuscinosis, neuronal 5 antikoerper, CLN5 antikoerper, Cln5 antikoerper

Hintergrund:

Synonyms: Ceroid lipofuscinosis neuronal 5, Ceroid-lipofuscinosis neuronal protein 5, CLN5, CLN5_HUMAN, NCL, Protein CLN5.

Background: Neuronal ceroid-lipofuscinose (NCL), also designated Batten disease, comprises a group of recessively inherited, progressive neurodegenerative diseases found in children. NCL is characterized by atrophy of the brain and an accumulation of lysosome derived fluorescent bodies found in many cells, especially neurons. Symptoms of NCL include a failure of psychomotor development, seizures, impaired vision and premature death. The eight genes/proteins associated with NCL are designated CLN1-CLN8. Mutations in six of these genes results in a distinct type of NCL-disease, the six genes/proteins are CLN1 (encoding PPT1, a protein thiolesterase), CLN2 (encodeing the serine protease TPP1), CLN3, CLN5, CLN6 and CLN8. A single base duplication mutation in dog and cow CLN5 has been shown to cause NCL.