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Rabbit Anti-CLN5/AF350 Conjugated antibody (bs-11714R-AF350)
订购热线:400-901-9800
订购邮箱:[email protected]
订购传真:010-58129612
技术支持:[email protected]
说 明 书:100ul  
100ul/2980.00元
大包装/询价
产品编号bs-11714R-AF350
英文名称Anti-CLN5/AF350
中文名称AF350标记的神经细胞蜡样质脂褐质沉积病蛋白CLN5抗体
别    名Ceroid lipofuscinosis neuronal 5; Ceroid-lipofuscinosis neuronal protein 5; CLN5; CLN5_HUMAN; NCL; Protein CLN5.  
规格价格100ul/2980元购买        大包装/询价
说 明 书100ul  
研究领域细胞生物  神经生物学  
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human, Mouse, Rat, Pig, Cow, Horse, Rabbit, Sheep, 
产品应用ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量37kDa
性    状Lyophilized or Liquid
浓    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human CLN5 (61-120aa)
亚    型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存条件Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
产品介绍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.

Subcellular Location:
Lysosome.

Tissue Specificity:
Ubiquitous.

Post-translational modifications:
Glycosylated.

DISEASE:
Defects in CLN5 are the cause of neuronal ceroid lipofuscinosis type 5 (CLN5) [MIM:256731]; also known as Finnish variant late-infantile neuronal ceroid lipofuscinosis (vLINCL). A form of neuronal ceroid lipofuscinosis. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. The lipopigment patterns observed most often in neuronal ceroid lipofuscinosis type 5 comprise mixed combinations of granular, curvilinear, and fingerprint profiles.

Similarity:
Belongs to the CLN5 family.

Database links:
UniProtKB/Swiss-Prot: O75503.2

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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