Product Name	Anti-NF-H/RBITC
Chinese Name	罗丹明（RBITC）标记的高分子量神经丝蛋白抗体
Alias	Neurofilament 200; 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; NFH; NFH_HUMAN; KIAA0845.
Research Area	Cell biology  Neurobiology  Signal transduction  Apoptosis  transcriptional regulatory factor
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Mouse,  (predicted: Human, Rat, )
Applications	IF=1:50-200  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Molecular weight	118kDa
Form	Lyophilized or Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human NF-H
Lsotype	IgG
Purification	affinity purified by Protein A
Storage Buffer	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	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.
Product Detail	background: 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. 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 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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