| 產(chǎn)品編號 | V5502 |
| 英文名稱 | NF-L |
| 中文名稱 | 低分子量神經(jīng)絲蛋白單克隆抗體 |
| 別 名 | Neurofilament L; Neurofilament 68; Neurofilament triplet L; 70 kD Neurofilament Light; 68kDa neurofilament protein; CMT 1F; CMT 2E; CMT1F; CMT2E; FLJ53642; Light molecular weight neurofilament protein; NEFL; Neurofilament light; Neurofilament light polypeptide 68kDa; Neurofilament light polypeptide; Neurofilament protein, light chain; Neurofilament subunit NF L; Neurofilament triplet L protein; NF 68; NF L; NF68; NFL; NFL_HUMAN. |
| 克 隆 號 | 3D11 |
| 理論分子量 | 68kDa |
| 細(xì)胞定位 | 細(xì)胞漿 |
| 性 狀 | Liquid |
| 緩 沖 液 | 0.01M PBS (pH7.4). |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 產(chǎn)品介紹 |
Neurofilament light polypeptide also called NF-L; Neurofilament triplet L protein; 68 kDa neurofilament protein. Neurofilaments usually contain three intermediate filament proteins: L, M, and H which are involved in the maintenance of neuronal caliber. The extra mass and high charge density that distinguish the neurofilament proteins from all other intermediate filament proteins are due to the tailpiece extensions. This region may form a charged scaffolding structure suitable for interaction with other neuronal components or ions. NF-L is the most abundant of the three neurofilament proteins and, as the other nonepithelial intermediate filament proteins, it can form homopolymeric 10-nm filaments. Belongs to the intermediate filament family. Function: Neurofilaments usually contain three intermediate filament proteins: L, M, and H which are involved in the maintenance of neuronal caliber. Subunit: Interacts with ARHGEF28. Interacts with TRIM2. Post-translational modifications: O-glycosylated. Phosphorylated in the head and rod regions by the PKC kinase PKN1, leading to the inhibition of polymerization. Ubiquitinated in the presence of TRIM2 and UBE2D1. DISEASE: Defects in NEFL are the cause of Charcot-Marie-Tooth disease type 1F (CMT1F) [MIM:607734]. CMT1F is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathy or CMT1, and primary peripheral axonal neuropathy or CMT2. Neuropathies of the CMT1 group are characterized by severely reduced nerve conduction velocities (less than 38 m/sec), segmental demyelination and remyelination with onion bulb formations on nerve biopsy, slowly progressive distal muscle atrophy and weakness, absent deep tendon reflexes, and hollow feet. CMT1F is characterized by onset in infancy or childhood (range 1 to 13 years). Defects in NEFL are the cause of Charcot-Marie-Tooth disease type 2E (CMT2E) [MIM:607684]. CMT2E is an autosomal dominant form of Charcot-Marie-Tooth disease type 2. Neuropathies of the CMT2 group are characterized by signs of axonal regeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy. Similarity: Belongs to the intermediate filament family. SWISS: P07196 Gene ID: 4747 Database links: Entrez Gene: 4747 Human Entrez Gene: 18039 Mouse Omim: 162280 Human SwissProt: P07196 Human SwissProt: P08551 Mouse Unigene: 521461 Human Unigene: 1956 Mouse Unigene: 18568 Rat 神經(jīng)生物學(xué)相關(guān)蛋白（Neurobiology） 低分子量神經(jīng)絲蛋白,簡稱NF-L,分子量為68kDa，NF-L的聚集與神經(jīng)退行性疾病的發(fā)病機(jī)理相關(guān)，如運(yùn)動神經(jīng)元的降解等。 神經(jīng)纖絲蛋白的功能是提供彈性使神經(jīng)纖維易于伸展和防止斷裂。 神經(jīng)絲是中間纖維的一種重要類型又稱神經(jīng)微絲蛋白，特異地在神經(jīng)細(xì)胞內(nèi)表達(dá)，并在軸突內(nèi)相互平行排列成束. 哺乳動物的神經(jīng)絲由3種蛋白組成： 低分子量神經(jīng)絲蛋白，簡稱NF-L；分子量為68kDa； 中分子量神經(jīng)絲蛋白，簡稱NF-M；分子量為160kDa； 高分子量神經(jīng)絲蛋白，簡稱NF-H，分子量為200 kDa。 |
| 產(chǎn)品圖片 |
Sample:
Lane 1: Recombinant Human NF-L Protein at 100ng
Primary: Mouse Anti-NF-L Monoclonal Antibody at 1/1000 dilution (Cat.V5502)
Secondary: IRDye800CW Goat Anti-Mouse IgG at 1/20000 dilution
Predicted band size: 61.4kD
Observed band size: 68/44kD
Measured by its binding ability in a indirect ELISA. Immobilized Human NF-L protein, His Tag (Cat. bs-41217P) at 2 μg/mL (100 μL/well) can bind ?Mouse Anti-Human NF-L Antibody, the EC50 is 24.94 ng/mL.
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