CLN6 Antikörper (AA 101-200) (Biotin)

Produktnummer ABIN891386

Produktdetails anti-CLN6 Antikörper (Biotin)

Target: CLN6 (Ceroid-Lipofuscinosis, Neuronal 6, Late Infantile, Variant (CLN6))

Bindungsspezifität: AA 101-200

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser CLN6 Antikörper ist konjugiert mit Biotin

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

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

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human CLN6

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 CLN6

Target: CLN6 (Ceroid-Lipofuscinosis, Neuronal 6, Late Infantile, Variant (CLN6))

Andere Bezeichnung: CLN6 (CLN6 Produkte)

Synonyme: 1810065L06Rik antikoerper, AW743417 antikoerper, D9Bwg1455e antikoerper, nclf antikoerper, CLN4A antikoerper, HsT18960 antikoerper, cln6 antikoerper, zgc:103565 antikoerper, ceroid-lipofuscinosis, neuronal 6 antikoerper, CLN6, transmembrane ER protein antikoerper, CLN6, transmembrane ER protein S homeolog antikoerper, ceroid-lipofuscinosis, neuronal 6, late infantile, variant antikoerper, CLN6, transmembrane ER protein a antikoerper, Cln6 antikoerper, CLN6 antikoerper, cln6.S antikoerper, cln6a antikoerper

Hintergrund:

Synonyms: Ceroid lipofuscinosis, neuronal 6, late infantile, variant, CLN6 protein, FLJ20561, Nclf, Protein CLN6, CLN6_HUMAN.

Background: CLN6, a 311 amino acid protein, has seven predicted transmembrane domains and is conserved across vertebrates. The CLN6 protein localizes to the endoplasmic reticulum but contributes to lysosomal function. Mutations in the CLN6 gene cause variant late-onset infantile neuronal ceroid lipofuscinosis (vLINCL), a lysosomal storage disorder marked by progressive mental deterioration and blindness, part of a group of severe inherited neurodegenerative disorders affecting children wherein lysosomes accumulate storage material, causing the death of neurons. CLN6 is one of eight proteins, including CLN1-8, that are associated with NCL.

Gen-ID: 54982

UniProt: Q9NWW5

Pathways: Glycosaminoglycan Metabolic Process