神经细胞蜡样质脂褐质沉积病蛋白CLN8抗体

Quantity size:

0.2ml

Concentration:

1mg/ml 神经细胞蜡样质脂褐质沉积病蛋白CLN8抗体  Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Background:

CLN8, a 286 amino acid transmembrane protein, localizes mainly to the endoplasmic reticulum,神经细胞蜡样质脂褐质沉积病蛋白CLN8抗体 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,神经细胞蜡样质脂褐质沉积病蛋白CLN8抗体 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.

Also known as:

神经细胞蜡样质脂褐质沉积病蛋白CLN8抗体Ceroid-lipofuscinosis, neuronal 8 (epilepsy, progressive with mental retardation); Cln8; CLN8_HUMAN; EPMR; Protein CLN8.

Specificity:

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Rabbit Polyclonal IgG, affinity purified by Protein A.

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Reacts with: Human, Mouse, Rat, Dog, Pig, Horse, Rabbit, .

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Immunogen: KLH conjugated synthetic peptide derived from human CLN8 (201-286aa).

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神经细胞蜡样质脂褐质沉积病蛋白CLN8抗体Predicted Molecular Weight: 33kDa.

Storage:

Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).

Application:

ELISA=1:500-1000  IHC-P=1:100-500  IHC-F=1:100-500  ICC=1:100-500  IF=1:100-500   
Not yet tested in other applications. 
Optimal working dilutions must be determined by the end user.

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