CLN6 anticorps (AA 101-200)

N° du produit ABIN872724

Détail du produit anti-CLN6 anticorps

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

Épitope: AA 101-200

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp CLN6 est non-conjugé

Application: Western Blotting (WB), ELISA, Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))

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

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human CLN6

Isotype: IgG

Information d'application

Indications d'application: WB 1:300-5000
ELISA 1:500-1000
IHC-P 1:200-400
IHC-F 1:100-500
IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 1 μg/μL

Buffer: 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % 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: 4 °C,-20 °C

Stockage commentaire: Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

Date de péremption: 12 months

Détails sur CLN6

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

Autre désignation: CLN6 (CLN6 Produits)

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

Sujet:

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.

ID gène: 54982

UniProt: Q9NWW5

Pathways: Glycosaminoglycan Metabolic Process