货号:ABN76
品牌:Merck Millipore
规格:100Ug
目录价:¥4967.00
市场价格:¥4221.95
会员价格:¥3973.60
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Description:
Anti-Neurofilament H (200 kDa) Antibody | ABN76
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Replaces:
AB1982
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Promotional Text:
Special Shipping Offer on Antibodies100% Performance Guaranteed
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Molecular Weight:
~200 kDa observed
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Immunogen:
GST-tagged recombinant protein corresponding to bovine Neurofilament H.
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Background Information:
Neurofilaments are a type of intermediate filament that serve as major elements of the cytoskeleton supporting the axon cytoplasm. They are the most abundant fibrillar components of the axon, being on average 3-10 times more frequent than axonal microtubules. Neurofilaments (10nm in dia.) are built from three intertwined protofibrils which are themselves composed of two tetrameric protofilament complexes of monomeric proteins. The neurofilament triplet proteins (68/70, 160, and 200 kDa) occur in both the central and peripheral nervous system and are usually neuron specific. The 68/70 kDa NF-L protein can self-assemble into a filamentous structure, however the 160 kDa NF-M and 200 kDa NF-H proteins require the presence of the 68/70 kDa NF-L protein to co-assemble. Neuromas, ganglioneuromas, gangliogliomas, ganglioneuroblastomas and neuroblastomas stain positively for neurofilaments. Although typically restricted to neurons, neurofilaments have been detected in paragangliomas, adrenal and extra-adrenal pheochromocytomas, carcinoids, neuroendocrine carcinomas of the skin, and oat cell carcinomas of the lung. For moreinformation, see the Nervous System Cell Type Specific Marker chart on the Millipore website.
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Species Reactivity:
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Human
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Mouse
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Bovine
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Rat
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Species Reactivity Note:
Demonstrated to react with Human, Mouse, Bovine, and Rat.
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Application Notes:
Immunohistochemistry Analysis: A 1 µg/mL dilution from a representative lot detected Neurofilament H in normal rat cerebellum tissue.
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Control:
Bovine cerebellum tissue lysate
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Quality Assurance:
Evaluated by Western Blot in bovine cerebellum tissue lysate.Western Blot Analysis: 0.1 µg/mL of this antibody detected Neurofilament H in bovine cerebellum tissue lysate.
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Purification Method:
Affinity purified
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Presentation:
Purified rabbit polyclonal in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide.
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Storage Conditions:
Stable for 1 year at 2-8°C from date of receipt.
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UniProt Number:
P12036
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Entrez Gene Number:
NP_066554.2
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Gene Symbol:
NEFH
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Alternate Names:
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Neurofilament heavy polypeptide
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Neurofilament H
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Usage Statement:
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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Key Applications:
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Western Blotting
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Immunohistochemistry (Paraffin)
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Entrez Gene Summary:
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.
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UniProt Summary:
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-TRANSLATION MODIFICATION: Modification 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.POLYMORPHISM: The number of repeats is shown to vary between 29 and 30.INVOLVEMENT IN 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.SEQUENCE SIMILARITIES: Belongs to the intermediate filament family.SEQUENCE CAUTION: The sequence BAA74868.2 differs from that shown. Reason: Erroneous initiation. The sequence BAG63896.1 differs from that shown. Reason: Erroneous initiation.
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Product Name:
Anti-Neurofilament H (200 kDa)
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Concentration:
1.0 mg/mL
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Antibody Type:
Polyclonal Antibody
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Qty/Pk:
100 μg
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Format:
Affinity Purified
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Host:
Rabbit
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