| 產(chǎn)品編號(hào) | bs-8016R |
| 英文名稱(chēng) | CLN3 Rabbit pAb |
| 中文名稱(chēng) | 神經(jīng)細(xì)胞蠟樣質(zhì)脂褐質(zhì)沉積病蛋白CLN3抗體 |
| 別 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. |
| 研究領(lǐng)域 | 細(xì)胞生物 神經(jīng)生物學(xué) |
| 抗體來(lái)源 | Rabbit |
| 克隆類(lèi)型 | Polyclonal |
| 克 隆 號(hào) | |
| 交叉反應(yīng) | Mouse (predicted: Human,Rat,Rabbit,Cow,Horse,Monkey,Macaque) |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 48 kDa |
| 檢測(cè)分子量 | |
| 細(xì)胞定位 | 細(xì)胞漿 細(xì)胞膜 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human CLN3: 75-140/438 |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
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 |
| 產(chǎn)品圖片 |
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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