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产品中心-北京博奥森生物技术有限公司
Recombinant Human CLN5 Protein, N-His (bs-104226P)  
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20ug/980.00元
50ug/1880.00元
100ug/2680.00元
大包装/询价
产品编号 bs-104226P
英文名称 Recombinant Human CLN5 Protein, N-His
中文名称
别    名 Protein CLN5; Ceroid-lipofuscinosis neuronal protein 5; CLN5  
克 隆 号
理论分子量 33.84 kDa
性    状 Lyophilized
纯    度 >90% as determined by SDS-PAGE.
缓 冲 液 Lyophilized from a solution in PBS pH 7.4, 0.02% NLS, 1mM EDTA, 4% Trehalose, 1% Mannitol.
保存条件 Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8°C for frequent use. Store at -20 to -80°C for twelve months from the date of receipt.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
产品介绍 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.

SWISS:
O75503

Gene ID:
1203

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