CLN6 Antikörper

Produktnummer ABIN317825

Produktdetails anti-CLN6 Antikörper

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

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser CLN6 Antikörper ist unkonjugiert

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

Spezifität: CLN6 antibody detects endogenous levels of CLN6 protein.(region surrounding asn258)

Aufreinigung: Affinity chromatography

Anwendungsinformationen

Applikationshinweise: ELISA: 1: 40000approx. 1: 60000. WB: 1: 500approx. 1: 1000. IHC: 1: 50approx. 1: 200.
Other applications not tested.
Optimal dilutions are dependent on conditions and should be determined by the user.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Konzentration: 1,0 mg/mL

Buffer: Phosphate buffered saline (PBS) with 0.05 % sodium azide, approx. pH 7.2.

Konservierungsmittel: Sodium azide

Vorsichtsmaßnahmen: This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Handhabung: Avoid repeated freezing and thawing.

Lagerung: 4 °C/-20 °C

Informationen zur Lagerung: Store the antibody undiluted at 2-8 °C for one month or (in aliquots) at -20 °C for longer.

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: CLN6, a 311-amino acid protein, has 7 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.Synonyms: Ceroid-lipofuscinosis neuronal protein 6

Molekulargewicht: approx. 40 kDa

Gen-ID: 54982

NCBI Accession: NP_060352

UniProt: Q9NWW5

Pathways: Glycosaminoglycan Metabolic Process