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NF-H Recombinant Rabbit mAb (bsm-52851R)  
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產品編號 bsm-52851R
英文名稱 NF-H Recombinant Rabbit mAb
中文名稱 高分子量神經絲蛋白抗體
別    名 NFH_HUMAN; Neurofilament heavy polypeptide; NEFH; KIAA0845; NFH; NF H; NF200; NF-200; Neurofilament 200; 200 kDa neurofilament protein; Neurofilament triplet H protein; Neurofilament H; Neurofilament heavy polypeptide 200kD;  
研究領域 細胞生物  神經生物學  信號轉導  細胞凋亡  轉錄調節因子  
抗體來源 Rabbit
克隆類型 Recombinant
克 隆 號 4C3
交叉反應 Human,Mouse,Rat
產品應用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 118 kDa
檢測分子量 200
細胞定位 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human NEFH: 690-740/1026 
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 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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 coincident with a change in the neurofilament function.
Phosphorylated in the Head and Rod regions by the PKC kinase PKN1, leading to inhibit 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.

SWISS:
P12036

Gene ID:
4744

Database links:

Entrez Gene: 4744 Human

Entrez Gene: 380684 Mouse

Entrez Gene: 24587 Rat

Omim: 162230 Human

SwissProt: P12036 Human

SwissProt: P19246 Mouse

SwissProt: P16884 Rat

Unigene: 198760 Human

Unigene: 298283 Mouse

Unigene: 108194 Rat



產品圖片
25 ug total protein per lane of various lysates (see on figure) probed with NF-H monoclonal antibody, unconjugated (bsm-52851R) at 1:1000 dilution and 4°C overnight incubation. Followed by conjugated secondary antibody incubation at r.t. for 60 min.
Paraformaldehyde-fixed, paraffin embedded Human Cerebellum; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with NF-H Monoclonal Antibody, Unconjugated(bsm-52851R) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
Paraformaldehyde-fixed, paraffin embedded Human Glioma; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with NF-H Monoclonal Antibody, Unconjugated(bsm-52851R) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
Paraformaldehyde-fixed, paraffin embedded Rat Cerebellum; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with NF-H Monoclonal Antibody, Unconjugated(bsm-52851R) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
Paraformaldehyde-fixed, paraffin embedded Human Cerebrum; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with NF-H Monoclonal Antibody, Unconjugated(bsm-52851R) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
Paraformaldehyde-fixed, paraffin embedded Rat Cerebrum; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with NF-H Monoclonal Antibody, Unconjugated(bsm-52851R) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
Paraformaldehyde-fixed, paraffin embedded Mouse Cerebrum; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with NF-H Monoclonal Antibody, Unconjugated(bsm-52851R) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
Paraformaldehyde-fixed, paraffin embedded Rat Spinal Cord; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with NF-H Monoclonal Antibody, Unconjugated(bsm-52851R) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
Paraformaldehyde-fixed, paraffin embedded Mouse Spinal Cord; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with NF-H Monoclonal Antibody, Unconjugated(bsm-52851R) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
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