CLN8 anticorps (AA 201-286) (Biotin)

N° du produit ABIN1392913

Détail du produit anti-CLN8 anticorps (Biotin)

Antigène: CLN8 (Ceroid-Lipofuscinosis, Neuronal 8 (Epilepsy, Progressive with Mental Retardation) (CLN8))

Épitope: AA 201-286

Reactivité: Humain, Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp CLN8 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, Rat

Homologie: Mouse,Dog,Pig,Horse,Rabbit

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human CLN8

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 CLN8

Antigène: CLN8 (Ceroid-Lipofuscinosis, Neuronal 8 (Epilepsy, Progressive with Mental Retardation) (CLN8))

Autre désignation: CLN8 (CLN8 Produits)

Synonymes: anticorps mnd, anticorps C8orf61, anticorps EPMR, anticorps CLN8, transmembrane ER and ERGIC protein, anticorps ceroid-lipofuscinosis, neuronal 8, anticorps CLN8, anticorps Cln8

Sujet:

Synonyms: Ceroid-lipofuscinosis, neuronal 8 epilepsy, progressive with mental retardation, Cln8, CLN8_HUMAN, EPMR, Protein CLN8, C8orf61

Background: CLN8, a 286 amino acid transmembrane protein, localizes mainly to the endoplasmic reticulum, but also partially to the ER-Golgi intermediate compartment (ERGIC). Mutations in the CLN8 gene cause neuronal ceroid lipofuscinosis 8 and progressive epilepsy with mental retardation (EPMR). Both disorders are forms of neuronal ceroid-lipofuscinose (NCL), a group of progressive neurodegenerative diseases found in children, characterized by failure of psychomotor development, impaired vision, seizures and premature death. The CLN8 protein is one of eight proteins in the CLN family, including CLN1-CLN7, which are associated with NCL.

ID gène: 2055

UniProt: Q9UBY8

Pathways: Regulation of Cell Size, Dicarboxylic Acid Transport