CLN5 antibody (AA 61-120) (AbBy Fluor® 680)

Catalog No. ABIN5000213

Product Details anti-CLN5 Antibody (AbBy Fluor® 680)

Target: CLN5 (Ceroid-Lipofuscinosis, Neuronal 5 (CLN5))

Binding Specificity: AA 61-120

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This CLN5 antibody is conjugated to AbBy Fluor® 680

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

Cross-Reactivity: Human

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

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human CLN5

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 CLN5

Target: CLN5 (Ceroid-Lipofuscinosis, Neuronal 5 (CLN5))

Alternative Name: CLN5 (CLN5 Products)

Synonyms: NCL antibody, A730075N08Rik antibody, CLN5, intracellular trafficking protein antibody, ceroid-lipofuscinosis, neuronal 5 antibody, CLN5 antibody, Cln5 antibody

Background:

Synonyms: Ceroid lipofuscinosis neuronal 5, Ceroid-lipofuscinosis neuronal protein 5, CLN5, CLN5_HUMAN, NCL, Protein CLN5.

Background: Neuronal ceroid-lipofuscinose (NCL), also designated Batten disease, comprises a group of recessively inherited, progressive neurodegenerative diseases found in children. NCL is characterized by atrophy of the brain and an accumulation of lysosome derived fluorescent bodies found in many cells, especially neurons. Symptoms of NCL include a failure of psychomotor development, seizures, impaired vision and premature death. The eight genes/proteins associated with NCL are designated CLN1-CLN8. Mutations in six of these genes results in a distinct type of NCL-disease, the six genes/proteins are CLN1 (encoding PPT1, a protein thiolesterase), CLN2 (encodeing the serine protease TPP1), CLN3, CLN5, CLN6 and CLN8. A single base duplication mutation in dog and cow CLN5 has been shown to cause NCL.