| 产品编号 | bs-8016R |
| 英文名称 | CLN3 Rabbit pAb |
| 中文名称 | 神经细胞蜡样质脂褐质沉积病蛋白CLN3抗体 |
| 别 名 | Batten disease protein; Battenin; BTS; Ceroid lipofuscinosis neuronal 3; Ceroid lipofuscinosis neuronal 3 juvenile(Batten Spielmeyer Vogt disease); Ceroid lipofuscinosis neuronal 3 juvenile; CLN 3; MGC102840; Protein CLN3; CLN3_HUMAN. |
| 研究领域 | 细胞生物 神经生物学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 克 隆 号 | |
| 交叉反应 | Mouse, (predicted: Human, Rat, Cow, Horse, Rabbit, Monkey, macaque) |
| 产品应用 | WB=1:500-2000 ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理论分子量 | 48 kDa |
| 检测分子量 | |
| 细胞定位 | 细胞浆 细胞膜 |
| 性 状 | Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human CLN3: 75-140/438 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 缓 冲 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存条件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 产品介绍 |
This gene encodes a protein that is involved in lysosomal function. Mutations in this, as well as other neuronal ceroid-lipofuscinosis (CLN) genes, cause neurodegenerative diseases commonly known as Batten disease or collectively known as neuronal ceroid lipofuscinoses (NCLs). Many alternatively spliced transcript variants have been found for this gene. Function: Involved in microtubule-dependent, anterograde transport of late endosomes and lysosomes. Subunit: Interacts with DCTN1 and KIF3A. Interacts with RAB7A and RILP. Subcellular Location: Lysosome membrane; Multi-pass membrane protein. Late endosome Post-translational modifications: Highly glycosylated. Farnesylation is important for trafficking to lysosomes. DISEASE: Defects in CLN3 are the cause of neuronal ceroid lipofuscinosis type 3 (CLN3) [MIM:204200]; also known as Batten disease. 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 hallmark of CLN3 is the ultrastructural pattern of lipopigment with a fingerprint profile, which can have 3 different appearances: pure within a lysosomal residual body; in conjunction with curvilinear or rectilinear profiles; and as a small component within large membrane-bound lysosomal vacuoles. The combination of fingerprint profiles within lysosomal vacuoles is a regular feature of blood lymphocytes from patients with CLN3. Similarity: Belongs to the battenin family. SWISS: Q13286 Gene ID: 1201 Database links: Entrez Gene: 1201 Human Entrez Gene: 12752 Mouse Omim: 607042 Human SwissProt: Q13286 Human SwissProt: Q61124 Mouse Unigene: 534667 Human Unigene: 268930 Mouse |
| 产品图片 |
Sample: Bone (Mouse) Lysate at 40 ug
Primary: Anti-CLN3 (bs-8016R) at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 48 kD
Observed band size: 50 kD
Sample:
Heart (Mouse) Lysate at 40 ug
Primary: Anti- CLN3 (bs-8016R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 48 kD
Observed band size: 50 kD
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| 1、抗体溶解方法 | |
| 2、抗体修复方式 | |
| 3、常用试剂的配制 | |
| 4、免疫组化操作步骤 | |
| 5、免疫组化问题解答 | |
| 6、Western Blotting 操作步骤 | |
| 7、Western Blotting 问题解答 | |
| 8、关于肽链的设计 | |
| 9、多肽的溶解与保存 | |
| 10、酶标抗体效价测定程序 | |
