产品名称
NF-L Rabbit Polyclonal Antibody
别名
NEFL; NF68; NFL; Neurofilament light polypeptide; NF-L; 68 kDa neurofilament protein; Neurofilament triplet L protein
蛋白名称
Neurofilament light polypeptide
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=4747
Human Swissprot No.
P07196
Human Swissprot Link
http://www.uniprot.org/uniprotkb/P07196/entry
Mouse Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=18039
Mouse Swissprot No.
P08551
Mouse Swissprot Link
http://www.uniprot.org/uniprot/P08551
Rat Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=83613
Rat Swissprot Link
http://www.uniprot.org/uniprot/P19527
免疫原
Synthesized peptide derived from the C-terminal region of human NF-L.
特异性
NF-L Polyclonal Antibody detects endogenous levels of NF-L protein.
稀释度
WB 1:500 - 1:2000. IHC-p: 1:100-300 ELISA: 1:40000.. IF 1:50-200
宿主
Polyclonal, Rabbit,IgG
背景介绍
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],
组织表达
Amygdala,Brain,Fetal brain cortex,Thalamus,
细胞定位
Cell projection, axon . Cytoplasm, cytoskeleton .
信号通路
Amyotrophic lateral sclerosis (ALS);
功能
caution: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.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.