高分子量神经丝蛋白抗体

Rrmab®兔单抗
2026-01-04~2026-02-28,RR26012026-01-04~2026-02-28,TR
高分子量神经丝蛋白抗体
货号:bsm-52851R
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概述

产品编号
bsm-52851R
产品类型
重组兔单抗、mIHC精品抗体
英文名称
NF-H Recombinant Rabbit mAb
中文名称
高分子量神经丝蛋白抗体
英文别名
CMT2CC; NFH; NF-H; NF200; mKIAA0845; NFH_HUMAN; NEFH; 200 kDa neurofilament protein; Neurofilament triplet H protein; KIAA0845; NFH_MOUSE; NFH_RAT;
抗体来源
Rabbit
免疫原
A synthesized peptide derived from human Neurofilament heavy polypeptide: 580-680
亚型
IgG
性状
Liquid
纯化方法
affinity purified by Protein A
克隆类型
Recombinant
克隆号
4C3
理论分子量
118 kDa
检测分子量
200 kDa
浓度
1mg/ml
储存液
0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
SWISS
Gene ID
保存条件
Shipped at 4℃. Store at -20℃ 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.
背景资料
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.
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产品应用

应用已检合格种属预测种属推荐稀释比例
WBRatHuman, Mouse1:500-2000
IHC-PHuman, Mouse, Rat1:100-500
IHC-FHuman, Mouse, Rat1:100-500
IFHuman, Mouse, Rat1:100-500

交叉反应

交叉反应: Human, Mouse, Rat

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靶标

基因名
NEFH
蛋白名
Neurofilament heavy polypeptide
翻译后修饰
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.
疾病
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.
相似性
Belongs to the intermediate filament family.
功能
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.

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