CLN8 Antikörper (AA 201-286) (Biotin)

Produktnummer ABIN1392913

Produktdetails anti-CLN8 Antikörper (Biotin)

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

Bindungsspezifität: AA 201-286

Reaktivität: Human, Ratte

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser CLN8 Antikörper ist konjugiert mit Biotin

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

Kreuzreaktivität: Human, Ratte

Homologie: Mouse,Dog,Pig,Horse,Rabbit

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human CLN8

Isotyp: IgG

Anwendungsinformationen

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

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 for 12 months.

Haltbarkeit: 12 months

Details zu CLN8

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

Andere Bezeichnung: CLN8 (CLN8 Produkte)

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

Hintergrund:

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.

Gen-ID: 2055

UniProt: Q9UBY8

Pathways: Regulation of Cell Size, Dicarboxylic Acid Transport