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NF-L Rabbit pAb (bs-0707R)  
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產(chǎn)品編號(hào) bs-0707R
英文名稱 NF-L Rabbit pAb
中文名稱 低分子量神經(jīng)絲蛋白抗體
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.  
研究領(lǐng)域 細(xì)胞生物  神經(jīng)生物學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  
抗體來(lái)源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Human,Mouse,Rat
產(chǎn)品應(yīng)用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,Flow-Cyt=1ug/Test
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 68 kDa
檢測(cè)分子量
細(xì)胞定位 細(xì)胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human NH-L intermedial: 301-400/543 
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 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

Entrez Gene: 83613 Rat

Omim: 162280 Human

SwissProt: P07196 Human

SwissProt: P08551 Mouse

SwissProt: P19527 Rat

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(jiǎn)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(dòng)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(dòng)物的神經(jīng)絲由3種蛋白組成:
低分子量神經(jīng)絲蛋白,簡(jiǎn)稱NF-L;分子量為68kDa;
中分子量神經(jīng)絲蛋白,簡(jiǎn)稱NF-M;分子量為160kDa;
高分子量神經(jīng)絲蛋白,簡(jiǎn)稱NF-H,分子量為200 kDa。
產(chǎn)品圖片
Sample: Lane 1: Mouse Cerebrum tissue lysates Lane 2: Human U251 cell lysates Primary: Anti-NF-L (bs-0707R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 68 kDa Observed band size: 68 kDa
Tissue/cell: rat brain tissue; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: 0.4% Pepsin, 37°C, 30min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-NF-L/Neurofilament L/Neurofilament 68 Polyclonal Antibody, Unconjugated (bs-0707R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
Tissue/cell: rat brain tissue; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-NF-L/Neurofilament L/Neurofilament 68 Polyclonal Antibody, Unconjugated (bs-0707R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
Tissue/cell: rat brain tissue; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-NF-L/Neurofilament L/Neurofilament 68 Polyclonal Antibody, Unconjugated (bs-0707R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
Blank control: SHSY5Y. Primary Antibody (green line): Rabbit Anti-NF-L antibody (bs-0707R) Dilution: 1ug/Test; Secondary Antibody : Goat anti-rabbit IgG-FITC Dilution: 0.5ug/Test. Protocol The cells were fixed with 4% PFA (10min at room temperature)and then permeabilized with 90% ice-cold methanol for 20 min at -20℃.The cells were then incubated in 5%BSA to block non-specific protein-protein interactions for 30 min at room temperature .Cells stained with Primary Antibody for 30 min at room temperature. The secondary antibody used for 40 min at room temperature. Acquisition of 20,000 events was performed.
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