Mouse Neurofilament NF-L Monoclonal Antibody | anti-NEFL antibody

Neurofilament NF-L Antibody

Cat. Num. AAA415293

• Gene Names: NEFL; NFL; NF-L; NF68; CMT1F; CMT2E
• Applications: Immunofluorescence, Western Blot
• Purity: Affinity purified
• Synonyms: Neurofilament NF-L; Monoclonal Antibody; Neurofilament NF-L Antibody; anti-NEFL antibody

• Host: Mouse
• Clonality: Monoclonal
• Isotype: IgG2b
• Specificity: Human, cow, pig, mouse, rat and all other mammals.
• Purity/Purification: Affinity purified
• Form/Format: Liquid
• Concentration: 100ug/100ul (varies by lot)
• Sequence Length: 543

• Applicable Applications for anti-NEFL antibody: Immunofluorescence (IF), Western Blot (WB)
• Application Notes: For immunofluorescence, use MCA-7D1 diluted 1:50 to 1:250. For western blots try MCA-7D1 diluted 1:2500 to 1:5000.
• Immunogen: 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. NF-L is the neurofilament light or low molecular weight polypeptide and runs on SDS-PAGE gels at about 68kDa. Antibodies to NF-L are useful for identifying neuronal cells and their processes in tissue sections and in tissue culture. NF-L antibody can also be useful in the neurofilament accumulations seen in many neurological diseases, such as Lou Gehrig's disease and Alzheimer's disease. Mutations in the protein coding region of the human NF-L gene cause some forms of Charcot-Marie-Tooth disease (1).
• Storage Buffer: PBS, pH 7.4 with 0.02% sodium azide.
• Preparation and Storage: This product is stable for several weeks at 4 degree C as an undiluted liquid. Dilute only prior to immediate use. For extended storage, aliquot contents and freeze at -20 degree C or below. Avoid cycles of freezing and thawing. Expiration date is one (1) year from date of receipt.

Western Blot (WB)

References

Mersiyanova IV, Perepelov AV, Polyakov AV, Sitnikov VF, Dadali EL, Oparin RB, Petrin AN and Evgrafov OV. A new variant of Charcot-Marie-Tooth disease type 2 is probably the result of a mutation in the neurofilament-light gene. Am. J. Hum. Genet. 67:37-46, 2000.

NCBI and Uniprot Product Information

• NCBI GI #: 105990539
• NCBI GeneID: 4747
• NCBI Accession #: NP_006149.2
• NCBI GenBank Nucleotide #: NM_006158.4
• UniProt Accession #: P07196; Q16154; Q8IU72; B9ZVN2
• Molecular Weight: 61,517 Da
• NCBI Official Full Name: neurofilament light polypeptide
• NCBI Official Synonym Full Names: neurofilament, light polypeptide
• NCBI Official Symbol: NEFL
• NCBI Official Synonym Symbols: NFL; NF-L; NF68; CMT1F; CMT2E
• NCBI Protein Information: neurofilament light polypeptide; neurofilament subunit NF-L; neurofilament triplet L protein; neurofilament protein, light chain; neurofilament, light polypeptide 68kDa; light molecular weight neurofilament protein
• UniProt Protein Name: Neurofilament light polypeptide
• Protein Family: Neurofilament light polypeptide
• UniProt Gene Name: NEFL
• UniProt Synonym Gene Names: NF68; NFL; NF-L
• UniProt Entry Name: NFL_HUMAN

NCBI Description

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]

Uniprot Description

Function: Neurofilaments usually contain three intermediate filament proteins: L, M, and H which are involved in the maintenance of neuronal caliber.

Subunit structure: Interacts with ARHGEF28

By similarity. Interacts with TRIM2

By similarity.

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.

Post-translational modification: O-glycosylated

By similarity.Phosphorylated in the head and rod regions by the PKC kinase PKN1, leading to the inhibition of polymerization. Ref.9Ubiquitinated in the presence of TRIM2 and UBE2D1

By similarity.

Involvement in disease: Charcot-Marie-Tooth disease 1F (CMT1F) [MIM:607734]: A dominant demyelinating form of Charcot-Marie-Tooth disease, a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies (designated CMT1 when they are dominantly inherited) and primary peripheral axonal neuropathies (CMT2). Demyelinating neuropathies 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).Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.14 Ref.15 Ref.16Charcot-Marie-Tooth disease 2E (CMT2E) [MIM:607684]: A dominant axonal form of Charcot-Marie-Tooth disease, a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies (designated CMT1 when they are dominantly inherited) and primary peripheral axonal neuropathies (CMT2). 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. Nerve conduction velocities are normal or slightly reduced.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.11 Ref.12 Ref.13 Ref.15 Ref.17

Miscellaneous: NF-L is the most abundant of the three neurofilament proteins and, like the other nonepithelial intermediate filament proteins, it can form homopolymeric 10-nm filaments.

Sequence similarities: Belongs to the intermediate filament family.