CLN8 抗体
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北京 101111
Quick Overview for CLN8 抗体 (ABIN2459283)
抗原
See all CLN8 抗体适用
宿主
克隆类型
标记
应用范围
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纯化方法
- Antibody is purified by peptide affinity chromatography method.
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免疫原
- Antibody produced in rabbits immunized with a synthetic peptide corresponding a region of human CLN8.
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anti-Ceroid-Lipofuscinosis, Neuronal 8 (Epilepsy, Progressive with Mental Retardation) (CLN8) (AA 201-286) antibody
CLN8 适用: 人, 大鼠 WB, ELISA, IF (cc), IF (p), IHC (fro), IHC (p), ICC 宿主: 兔 Polyclonal unconjugated
anti-Ceroid-Lipofuscinosis, Neuronal 8 (Epilepsy, Progressive with Mental Retardation) (CLN8) (AA 247-286) antibodyCLN8 适用: 人 ELISA, IHC 宿主: 兔 Polyclonal unconjugated
anti-Ceroid-Lipofuscinosis, Neuronal 8 (Epilepsy, Progressive with Mental Retardation) (CLN8) (N-Term) antibodyCLN8 适用: 人, 犬, 马, 猴 WB 宿主: 兔 Polyclonal unconjugated
anti-Ceroid-Lipofuscinosis, Neuronal 8 (Epilepsy, Progressive with Mental Retardation) (CLN8) antibodyCLN8 适用: 人, 犬 WB 宿主: 兔 Polyclonal unconjugated
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应用备注
- CLN8 antibody can be used for detection of CLN8 by ELISA at 1:12500. CLN8 antibody can be used for detection of CLN8 by western blot at 1 μg/mL, and HRP conjugated secondary antibody should be diluted 1:50,000 - 100,000.
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限制
- 仅限研究用
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状态
- Lyophilized
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溶解方式
- Add 50 ?L of distilled water. Final antibody concentration is 1 mg/mL.
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浓度
- 1 mg/mL
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缓冲液
- Antibody is lyophilized in PBS buffer with 2 % sucrose.
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注意事项
- As with any antibody avoid repeat freeze-thaw cycles.
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储存条件
- 4 °C/-20 °C
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储存方法
- For short periods of storage (days) store at 4 °C. For longer periods of storage, store CLN8 antibody at -20 °C.
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- CLN8 (Ceroid-Lipofuscinosis, Neuronal 8 (Epilepsy, Progressive with Mental Retardation) (CLN8))
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别名
- CLN8
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背景
- CLN8 is a transmembrane protein belonging to a family of proteins containing TLC domains, which are postulated to function in lipid synthesis, transport, or sensing. The protein localizes to the endoplasmic reticulum (ER), and may recycle between the ER and ER-Golgi intermediate compartment. Mutations in this gene are associated with progressive epilepsy with mental retardation (EMPR), which is a subtype of neuronal ceroid lipofuscinoses (NCL). Patients with mutations in this gene have altered levels of sphingolipid and phospholipids in the brain. Childhood-onset NCL are a group of autosomal recessive progressive encephalopathies characterized by the accumulation of autofluorescent material, mainly ATP synthase subunit C, in various tissues, notably in neurons. Based on clinical features, the country of origin of patients, and the molecular genetic background of the disorder, at least seven different forms are thought to exist. CLN8 is characterized by normal early development, onset of generalized seizures between 5 and 10 years, and subsequent progressive mental retardation.This gene encodes a transmembrane protein belonging to a family of proteins containing TLC domains, which are postulated to function in lipid synthesis, transport, or sensing. The protein localizes to the endoplasmic reticulum (ER), and may recycle between the ER and ER-Golgi intermediate compartment. Mutations in this gene are associated with progressive epilepsy with mental retardation (EMPR), which is a subtype of neuronal ceroid lipofuscinoses (NCL). Patients with mutations in this gene have altered levels of sphingolipid and phospholipids in the brain.
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分子量
- 33 kDa
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基因ID
- 2055
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NCBI登录号
- NP_061764
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UniProt
- Q9UBY8
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途径
- Regulation of Cell Size, Dicarboxylic Acid Transport
抗原
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