神经细胞蜡样质脂褐质沉积病蛋白CLN5抗体

CLN5 Antibody

Cat#：PL0306720

Isotype:Rabbit Polyclonal IgG

Immunogen：Peptide derived from human CLN5 (61-120aa).

Purification: Immunogen affinity purified

Storage Buffer: PBS, pH 7.4(1%BSA and 0.1% Sodium azide)

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.

Specificity Reaction：Hu, Mo, Rat, Pig, Cow, Hor, , Sheep

Application Notes:WB: 1:100-500 E: 1:500-1000 IHC: 1:100-500 IF: 1:100-500 ICC: 1:100-500

Storage ：

Store at –20ºC for one year. Avoid repeated freeze-thaw cycles. At 4ºC for 2-3 weeks after reconstituted.

This product for research use only, not for use in humans, therapeutic or diagnostic procedures.

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