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高分子量神經(jīng)絲蛋白抗體規(guī)格 英文名稱  Anti-NF-H/Neurofilament H/Neurofilament 200/NF200

中文名稱  高分子量神經(jīng)絲蛋白抗體規(guī)格 

別    名 200 kDa neurofilament protein; 200 kD Neurofilament Heavy; NEFH; NEFH; NF200; NF-200; Neurofilament H; Neurofilament heavy polypeptide 200kD; Neurofilament heavy polypeptide 200kDa; Neurofilament heavy polypeptide; Neurofilament triplet H protein; Neurofilament triplet H protein; Hypophosphorylated Neurofilament H; NF H; NF-H; NFH; NFH_HUMAN; KIAA0845.

產(chǎn)品屬性：

濃    度  1mg/1ml

規(guī) 格  0.1ml/100μg 0.2ml/200μg

抗體來源  Rabbit

克隆類型   polyclonal

交叉反應   Human, Mouse, Rat

產(chǎn)品類型   一抗  

研究領(lǐng)域    細胞生物 神經(jīng)生物學 信號轉(zhuǎn)導 細胞凋亡 轉(zhuǎn)錄調(diào)節(jié)因子

蛋白分子量  predicted molecular weight: 118kDa 

性    狀   Lyophilized or Liquid

免 疫 原  KLH conjugated synthetic peptide derived from human NF-H N-terminus

亞    型  IgG

純化方法   affinity purified by Protein A

儲 存 液   0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide

高分子量神經(jīng)絲蛋白抗體規(guī)格 產(chǎn)品應用   WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500

（石蠟切片需做抗原修復） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

保存條件  Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. 

Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

產(chǎn)品介紹 Neurofilaments can be defined as the intermediate or 10nm filaments found in specifically in neuronal cells. When visualised using an electron microscope, neurofilaments appear as 10nm diameter fibres of indeterminate length that generally have fine wispy protrusions from their sides. They are particularly abundant in axons of large projection neurons. They probably function to provide structural support for neurons and their synapses and to support the large axon diameters required for rapid conduction of impulses down axons. Neurofilaments are composed of a mixture of subunits, which usually includes the three neurofilament triplet proteins neurofilament light (NFL), neurofilament medium (NFM) and neurofilament heavy (NFH). Neurofilaments may also include smaller amounts of peripherin, alpha internexin, nestin and in some cases vimentin. Antibodies to the various neurofilament subunits are very useful cell type markers since the proteins are among the most abundant of the nervous system, are expressed only in neurons, and are biochemically very stable. Some studies have shown that levels of neurofilament heavy and neurofilament light are elevated in patients with Alzheimer's disease, frontotemporal lobe dementia, and vascular dementia.

Function : Neurofilaments usually contain three intermediate filament proteins: L, M, and H which are involved in the maintenance of neuronal caliber. NF-H has an important function in mature axons that is not subserved by the two smaller NF proteins.Post-translational modifications : There are a number of repeats of the tripeptide K-S-P, NFH is phosphorylated on a number of the serines in this motif. It is thought that phosphorylation of NFH results in the formation of interfilament cross bridges that are important in the maintenance of axonal caliber.Phosphorylation seems to play a major role in the functioning of the larger neurofilament polypeptides (NF-M and NF-H), the levels of phosphorylation being altered developmentally and coincidentally with a change in the neurofilament function.Phosphorylated in the head and rod regions by the PKC kinase PKN1, leading to the inhibition of polymerization.DISEASE : Defects in NEFH are a cause of susceptibility to amyotrophic lateral sclerosis (ALS) [MIM:105400]. ALS is a neurodegenerative disorder affecting upper and lower motor neurons, and resulting in fatal paralysis. Sensory abnormalities are absent. Death usually occurs within 2 to 5 years. The etiology is likely to be multifactorial, involving both genetic and environmental factors.Similarity : Belongs to the intermediate filament family.Database links :Entrez Gene: 4744 HumanEntrez Gene: 380684 MouseEntrez Gene: 24587 RatOmim: 162230 HumanSwissProt: P12036 HumanSwissProt: P19246 MouseSwissProt: P16884 RatUnigene: 198760 Human神經(jīng)生物學相關(guān)蛋白（Neurobiology）高分子量神經(jīng)絲蛋白,簡稱NF-H,分子量為200kDa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)細胞內(nèi)表達，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200kDa；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。NF是神經(jīng)元特異性中間絲蛋白，由三個不同分子量的亞單位構(gòu)成的多聚體，以不同的比例分布于中樞、外周神經(jīng)元以及中，其陽性主要于節(jié)細胞神經(jīng)瘤、副節(jié)瘤、小腦或外周神經(jīng)母細胞瘤、腎上腺瘤或外周嗜鉻細胞瘤，有助于神經(jīng)母細胞瘤和嗜鉻細胞瘤的診斷與鑒別診斷。

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