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- 抗原 See all CLN8 抗体
- CLN8 (Ceroid-Lipofuscinosis, Neuronal 8 (Epilepsy, Progressive with Mental Retardation) (CLN8))
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抗原表位
- N-Term
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适用
- 人, 大鼠, 犬, 小鼠, 豚鼠, Cow, 兔
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宿主
- 兔
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克隆类型
- 多克隆
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标记
- This CLN8 antibody is un-conjugated
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应用范围
- Western Blotting (WB)
- 序列
- VFGVQSTAAG LWALLGDPVL HADKARGQQN WCWFHITTAT GFFCFENVAV
- 预测反应
- Cow: 79%, Dog: 100%, Guinea Pig: 86%, Human: 100%, Mouse: 86%, Rabbit: 79%, Rat: 86%
- 产品特性
- This is a rabbit polyclonal antibody against CLN8. It was validated on Western Blot using a cell lysate as a positive control.
- 纯化方法
- Affinity Purified
- 免疫原
- The immunogen is a synthetic peptide directed towards the N terminal region of human CLN8
- Top Product
- Discover our top product CLN8 Primary Antibody
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anti-Ceroid-Lipofuscinosis, Neuronal 8 (Epilepsy, Progressive with Mental Retardation) (CLN8) antibody
CLN8 适用: 人 WB, IHC 宿主: 兔 Polyclonal unconjugated
anti-Ceroid-Lipofuscinosis, Neuronal 8 (Epilepsy, Progressive with Mental Retardation) (CLN8) (AA 201-286) antibodyCLN8 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) (AA 72-121) antibodyCLN8 适用: 人, 犬, 小鼠, Cow WB 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
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- 应用备注
- Optimal working dilutions should be determined experimentally by the investigator.
- 说明
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Antigen size: 286 AA
- 限制
- 仅限研究用
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- 状态
- Liquid
- 浓度
- Lot specific
- 缓冲液
- Liquid. Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.
- 储存液
- Sodium azide
- 注意事项
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
- 注意事项
- Avoid repeated freeze-thaw cycles.
- 储存条件
- -20 °C
- 储存方法
- For short term use, store at 2-8°C up to 1 week. For long term storage, store at -20°C in small aliquots to prevent freeze-thaw cycles.
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- 抗原
- CLN8 (Ceroid-Lipofuscinosis, Neuronal 8 (Epilepsy, Progressive with Mental Retardation) (CLN8))
- 别名
- CLN8 (CLN8 产品)
- 别名
- mnd antibody, C8orf61 antibody, EPMR antibody, CLN8, transmembrane ER and ERGIC protein antibody, ceroid-lipofuscinosis, neuronal 8 antibody, CLN8 antibody, Cln8 antibody
- 背景
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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.
Alias Symbols: C8orf61, EPMR, FLJ39417
Protein Interaction Partner: YIPF6, ZUFSP, TMEM128, TMEM107, CYB5B, TMEM134, SELK, NDRG2, RTN4, RRP15, TMEM14A, GABARAPL2, C14orf1, ARL6IP5, TMEM147, TUBB4A, PDIA6, SPCS2, STX8, VAPA, TMEM11, UBC, PTPRN, PLP2, PLP1, MTX1, GPM6B, CD9, BNIP3L, BNIP3,
Protein Size: 286 - 分子量
- 33 kDa
- 基因ID
- 2055
- NCBI登录号
- NM_018941, NP_061764
- UniProt
- Q9UBY8
- 途径
- Regulation of Cell Size, Dicarboxylic Acid Transport
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