抗体

Anti-Neurofilament H (NF-H)

MBS500006

0.1毫升

235美元

单克隆

小鼠

未共轭

NAP4

人类, 大鼠

免疫印迹, 免疫细胞化学

抗体

Anti-Neurofilament H (NF-H)

Neurofilament NF-H ms

neurofilament heavy polypeptide; Neurofilament heavy polypeptide; neurofilament heavy polypeptide; NF-H; 200 kDa neurofilament protein; neurofilament triplet H protein; neurofilament, heavy polypeptide 200kDa; neurofilament, heavy polypeptide; 200 kDa neurofilament protein; Neurofilament triplet H protein

NEFH

NEFH;NEFH;NFH;KIAA0845;NFH;NF-H

NFH_HUMAN

单克隆

IgG1

NAP4

小鼠

大鼠

1020

Specific for the ~200k Neurofilament H protein. It recognizes phosphorylated NF-H KSP (lysine-serine-proline) type sequences. In some species there is some cross-reactivity with the related phosphorylated KSP sequences found in the related neurofilament subunit NF-M. It recognizes neurofilaments in frozen sections in tissue culture and in formalin fixed sections.

Total IgG fraction (Unpurified mouse ascites fluid.)

100 ul mouse ascites fluid

Store at -20 degree C in undiluted aliquots; stable for at least 1 year. Avoid freeze/thaw cycles.

免疫印迹(免疫印迹), 免疫荧光(IF)

Quality Control: Western blots performed on each lot. WB: 1:5,000. IF: 1:500

Antigen: Purified bovine neurofilament H. Immunogen Information: Purified bovine NF-H. Immunogen Species: Bovine

Reactivity Assumed Based on 100% Sequence Homology: Human, mouse. Species Reactivity Note: The antibody has been directly tested for reactivity in chicken and a wide variety of mammals. Biological Significance: Neurofilaments are the 10nm or intermediate filament proteins found specifically in neurons, and are composed predominantly of three major proteins called NF-L, NF-M and NF-H (1). NF-H is the neurofilament high or heavy molecular weight polypeptide and runs on SDS-PAGE gels at 200-220 kDa, with some variability across species boundaries. Antibodies to NF-H are useful for identifying neuronal cells and their processes in tissue sections and in tissue culture. NF-H antibodies can also be useful to visualize neurofilament accumulations seen in many neurological diseases, such as Amyotrophic Lateral Sclerosis (Lou Gehrig's disease) (2) and Alzheimer's disease (3).

小鼠单克隆抗体

Harris, J., Ayyub, C. and Shaw G. (1991) A molecular dissection of the carboxyterminal tails of the major neurofilament subunits NF-M and NF-H. J Neurosci Res 30:47-62. Mendonca DM, Chimelli L, Martinez AM. (2005) Quantitative evidence for neurofilament heavy subunit aggregation in motor neurons of spinal cords of patients with amyotrophic lateral sclerosis. Braz J Med Biol Res. 38(6):925-933. Hu YY, He SS, Wang XC, Duan QH, Khatoon S, Igbal K, Grundke-Igbal I, Wang JZ (2002) Elevated levels of phosphorylated neurofilament proteins in cerebrospinal fluid of Alzheimer disease patients. Neurosci Lett 320(3):156-60.

32483416

NP_066554.2

NM_021076.3

P12036

200

Amyotrophic Lateral Sclerosis (ALS) Pathway 920975!!Amyotrophic Lateral Sclerosis (ALS) Pathway 83099!!Amyotrophic Lateral Sclerosis (ALS) Pathway 511

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]

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 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. Ref.7Phosphorylation 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 coincidentally with a change in the neurofilament function.Phosphorylated in the head and rod regions by the PKC kinase PKN1, leading to the inhibition of polymerization. Ref.7. Polymorphism: The number of repeats is shown to vary between 29 and 30. Involvement in disease: Amyotrophic lateral sclerosis (ALS) [MIM:105400]: A neurodegenerative disorder affecting upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord, resulting in fatal paralysis. Sensory abnormalities are absent. The pathologic hallmarks of the disease include pallor of the corticospinal tract due to loss of motor neurons, presence of ubiquitin-positive inclusions within surviving motor neurons, and deposition of pathologic aggregates. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of the cases.Note: Disease susceptibility is associated with variations affecting the gene represented in this entry. Ref.8. 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.

0.1毫升

235美元