CLN8 antibody (AA 201-286) (Alexa Fluor 680)

Catalog No. ABIN5000217

Product Details anti-CLN8 Antibody (Alexa Fluor 680)

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

Binding Specificity: AA 201-286

Reactivity: Human, Rat

Host: Rabbit

Clonality: Polyclonal

Conjugate: This CLN8 antibody is conjugated to Alexa Fluor 680

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

Cross-Reactivity: Human, Rat

Predicted Reactivity: Mouse,Dog,Pig,Horse,Rabbit

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human CLN8

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 CLN8

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

Alternative Name: CLN8 (CLN8 Products)

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

Background:

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.

Gene ID: 2055

UniProt: Q9UBY8

Pathways: Regulation of Cell Size, Dicarboxylic Acid Transport