CLN6 antibody (AA 101-200) (Cy7)

Catalog No. ABIN891376

Product Details anti-CLN6 Antibody (Cy7)

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

Binding Specificity: AA 101-200

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This CLN6 antibody is conjugated to Cy7

Application: Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Predicted Reactivity: Human,Mouse,Rat,Cow,Sheep,Pig,Horse,Rabbit

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human CLN6

Isotype: IgG

Application Details

Application Notes: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Restrictions: For Research Use only

Handling

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.

Preservative: ProClin

Precaution of Use: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Storage: -20 °C

Storage Comment: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Expiry Date: 12 months

Target Details for CLN6

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

Alternative Name: CLN6 (CLN6 Products)

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

Background:

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.

Gene ID: 54982

UniProt: Q9NWW5

Pathways: Glycosaminoglycan Metabolic Process