SKU: WM1436 Categories: ,

Neurofilament [ABT356] mouse mAb

Price range: $268.00 through $328.00

SKU: WM1436-50
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Product details

Background:
Neurofilament (NF) is a neuron specific intermediate filament protein. It is a polymer composed of subunits of NF-H, NF-M and NF-L. It exists in neurons, neurites, peripheral nerve fibers, sympathetic ganglion cells and adrenal medulla. It is expressed in ganglioneuroma, paraganglioma, cerebellar or peripheral neuroblastoma, adrenal tumor or peripheral pheochromocytoma. It is helpful to the diagnosis and differential diagnosis of neuroblastoma and pheochromocytoma, and to identify the neurons of Hirschsprung’s disease.

Specifications

Size50μL/100μL
TargetNF-L
ReactivityHuman
ApplicationsIHC, WB, IF, ELISA
MW(Calculated)68kD
MW(Observed)68kD
Host SpeciesMouse
IsotypeIgG2b,Kappa
Conjugate/ModificationUnmodified
Recommended Dilution RatioIHC 1:200-1000; WB 1:500-2000; IF 1:100-500; ELISA 1:1000-5000 / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004)
FormulationPBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
PurificationRecombinant Antibody expressed in animal component-free (ACF) media, purified via Protein G affinity chromatography.
Storage-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration
ClonalityMonoclonal
Clone NumberRMM356
ImmunogenSynthesized peptide derived from human Neurofilament AA range: 400-543
SpecificityThe antibody can specifically recognize human Neurofilament protein, especilaly NF-L protein.
Gene NameNEFL NF68 NFL
Protein NameNeurofilament light polypeptide; NF-L; Neurofilament triplet L protein; 68 kDa neurofilament protein
Other NameNeurofilament light polypeptide; / NF-L; / 68 kDa neurofilament protein; / Neurofilament triplet L protein
SpeciesHuman
Gene ID4747
UniprotP07196,
Species.1Mouse
Gene ID.118039
Uniprot.1P08551,
Species.2Rat
Gene ID.283613
Uniprot.2P19527
Cellular LocalizationCytoplasmic
Tissue ExpressionCytoplasmic
Signaling_pathwayHuman Diseases >> Neurodegenerative disease >> Amyotrophic lateral sclerosis;Human Diseases >> Neurodegenerative disease >> Pathways of neurodegeneration - multiple diseases
Research Areas>>Amyotrophic lateral sclerosis; / >>Pathways of neurodegeneration - multiple diseases
FunctionCaution:The sequence shown here is derived from an Ensembl automatic analysis pipeline and should be considered as preliminary data.,Disease:Defects in NEFL are the cause of Charcot-Marie-Tooth disease type 1F (CMT1F) [MIM:607734]. CMT1F is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathy or CMT1, and primary peripheral axonal neuropathy or CMT2. Neuropathies of the CMT1 group are characterized by severely reduced nerve conduction velocities (less than 38 m/sec), segmental demyelination and remyelination with onion bulb formations on nerve biopsy, slowly progressive distal muscle atrophy and weakness, absent deep tendon reflexes, and hollow feet. CMT1F is characterized by onset in infancy or childhood (range 1 to 13 years).,Disease:Defects in NEFL are the cause of Charcot-Marie-Tooth disease type 2E (CMT2E) [MIM:607684]. CMT2E is an autosomal dominant form of Charcot-Marie-Tooth disease type 2. Neuropathies of the CMT2 group are characterized by signs of axonal regeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy.,Domain:The extra mass and high charge density that distinguish the neurofilament proteins from all other intermediate filament proteins are due to the tailpiece extensions. This region may form a charged scaffolding structure suitable for interaction with other neuronal components or ions.,Function:Neurofilaments usually contain three intermediate filament proteins: L, M, and H which are involved in the maintenance of neuronal caliber.,miscellaneous:NF-L is the most abundant of the three neurofilament proteins and, as the other nonepithelial intermediate filament proteins, it can form homopolymeric 10-nm filaments.,PTM:O-glycosylated.,similarity:Belongs to the intermediate filament family.,subunit:Interacts with RGNEF.,
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