SKU: WM1722R Categories: ,

U-Blot® Neurofilament(NF-L) Mouse mAb (Ready to Use)

$168.00

SKU: WM1722R
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Product details

Background:
Neurofilaments are type IV intermediate filament heteropolymers composed of light, medium, and heavy chains. Neurofilaments comprise the axoskeleton and they functionally maintain the neuronal caliber. They may also play a role in intracellular transport to axons and dendrites. This gene encodes the light chain neurofilament protein. Mutations in this gene cause Charcot-Marie-Tooth disease types 1F (CMT1F) and 2E (CMT2E), disorders of the peripheral nervous system that are characterized by distinct neuropathies. A pseudogene has been identified on chromosome Y. [provided by RefSeq, Oct 2008],

Specifications

TargetNF-L
ReactivityHuman
ApplicationsIHC
MW(Calculated)
MW(Observed)
Host SpeciesMouse
IsotypeIgG2b,Kappa
Conjugate/ModificationUnmodified
Modification
Recommended Dilution RatioReady to use for IHC / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#YS0004)
FormulationThe prediluted ready-to-use antibody is diluted in phosphate buffer saline containing stabilizing protein and 0.05% Proclin 300
Source
PurificationThe antibody was affinity-purified from ascites by affinity-chromatography using specific immunogen.
Purity
Storage2°C to 8°C/1 year,Ship by ice bag
Concentration
ClonalityMonoclonal
Clone NumberABT208
ImmunogenSynthesized peptide derived from human Neurofilament AA range: 400-543
Sequence
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 ID-14747
UniprotP07196,
Species.1Mouse
Gene ID-218039
Uniprot.1P08551,
Species.2Rat
Gene ID-383613
Uniprot.2P19527
Organism-4
Gene ID-4
SwissProt-4
BackgroundNeurofilaments are type IV intermediate filament heteropolymers composed of light, medium, and heavy chains. Neurofilaments comprise the axoskeleton and they functionally maintain the neuronal caliber. They may also play a role in intracellular transport to axons and dendrites. This gene encodes the light chain neurofilament protein. Mutations in this gene cause Charcot-Marie-Tooth disease types 1F (CMT1F) and 2E (CMT2E), disorders of the peripheral nervous system that are characterized by distinct neuropathies. A pseudogene has been identified on chromosome Y. [provided by RefSeq, Oct 2008],
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.,
RRID
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