CLN5 anticorps (AA 61-120) (Biotin)

N° du produit ABIN1392907

Détail du produit anti-CLN5 anticorps (Biotin)

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

Épitope: AA 61-120

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp CLN5 est conjugé à/à la Biotin

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

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

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 for 12 months.

Date de péremption: 12 months

Détails sur CLN5

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

Autre désignation: CLN5 (CLN5 Produits)

Synonymes: anticorps NCL, anticorps A730075N08Rik, anticorps CLN5, intracellular trafficking protein, anticorps ceroid-lipofuscinosis, neuronal 5, anticorps CLN5, anticorps 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.