免疫原
The antiserum was produced against synthesized peptide derived from human NF-H. AA range:923-972
特异性
NF-H Polyclonal Antibody detects endogenous levels of NF-H protein.
来源
Polyclonal, Rabbit,IgG
组成（Formulation）
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
稀释比例
IHC: 1/100 - 1/300. ELISA: 1/40000.. IF 1:50-200
纯化工艺（Immunogen）
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
背景（Background）
Neurofilaments are type IV intermediate filament heteropolymers composed of light, medium, and heavy chains. Neurofilaments comprise the axoskeleton and functionally maintain neuronal caliber. They may also play a role in intracellular transport to axons and dendrites. This gene encodes the heavy neurofilament protein. This protein is commonly used as a biomarker of neuronal damage and susceptibility to amyotrophic lateral sclerosis (ALS) has been associated with mutations in this gene. [provided by RefSeq, Oct 2008],
功能
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.,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.,online information:ALS genetic mutations db,polymorphism:The number of repeats is shown to vary between 29 and 30.,PTM:Phosphorylation seems to play a major role in the functioning of the larger neurofilament polypeptides (NF-M and NF-H), the levels of phosphor
蛋白名称
Neurofilament heavy polypeptide
其他名称
NEFH; KIAA0845; NFH; Neurofilament heavy polypeptide; NF-H; 200 kDa neurofilament protein; Neurofilament triplet H protein
Fields
>>Amyotrophic lateral sclerosis;>>Pathways of neurodegeneration - multiple diseases
细胞定位
Cytoplasm, cytoskeleton . Cell projection, axon .
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