| 供货周期： | 现货 |
| 品牌： | 康朗生物 |
| 规格： | 0.1ml/0.2ml |
| 货号： | KL11714R |
| CAS号： |
Quantity size:
0.2ml
Concentration:
1mg/ml 神经细胞蜡样质脂褐质沉积病蛋白CLN5抗体 Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Background:
Neuronal ceroid-lipofuscinose (NCL), also designated Batten disease, comprises a group of recessively inherited, progressive neurodegenerative diseases found in children.神经细胞蜡样质脂褐质沉积病蛋白CLN5抗体 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),神经细胞蜡样质脂褐质沉积病蛋白CLN5抗体 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.
Also known as:
Ceroid lipofuscinosis neuronal 5; Ceroid-lipofuscinosis neuronal protein 5; CLN5; CLN5_HUMAN; NCL; Protein CLN5.
Specificity:
●
神经细胞蜡样质脂褐质沉积病蛋白CLN5抗体Rabbit Polyclonal IgG, affinity purified by Protein A.
●
Reacts with: Human, Mouse, Rat, Pig, Cow, Horse, Rabbit, Sheep, .
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Immunogen: KLH conjugated synthetic peptide derived from human CLN5 (61-120aa).
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Predicted Molecular Weight: 37kDa.
Storage:
Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).
神经细胞蜡样质脂褐质沉积病蛋白CLN5抗体Application:
WB=1:100-500 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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