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

N° du produit ABIN1392904

Cet anticorps Lapin Polyclonal détecte spécifiquement CLN5 dans WB, IF (cc) et IF (p). Il présente une réactivité envers Humain.

Aperçu rapide pour CLN5 anticorps (AA 61-120) (AbBy Fluor® 488) (ABIN1392904)

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

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp CLN5 est conjugé à/à la AbBy Fluor® 488

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

Détail du produit anti-CLN5 anticorps (AbBy Fluor® 488)

Épitope: AA 61-120

Réactivité croisée: Humain

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

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human CLN5

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 CLN5

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

Autre désignation: CLN5

Sujet:

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.