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Rabbit Anti-NEFH  antibody (bsm-60885R)
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说明书: 50ul  100ul  
50ul/1580.00元
100ul/2500.00元
大包装/询价
产品编号 bsm-60885R
英文名称 Rabbit Anti-NEFH  antibody
中文名称 高分子量神经丝蛋白重组兔单抗
别    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;  
抗体来源 Rabbit
克隆类型 Recombinant
交叉反应 Human,Mouse,Rat
产品应用 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.
理论分子量 112kDa
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human NF-H 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 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:
P12036

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



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