| Description | |
|---|---|
| Catalogue Number | AG231 |
| Brand Family | Chemicon® |
| Trade Name |
|
| Description | Neurofilament 70 kDa, porcine |
| Overview | Purified porcine axonal neurofilament 70 (low molecular weight, NF-L). |
| Product Information | |
|---|---|
| Presentation | Liquid in 6M Urea, 10 mM Phosphate, pH 7.5. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Key Applications |
|
| Application Notes | Western blotting Protein standard ELISA Radioimmunoassay Optimal working dilution must be determined by the end user. |
| Biological Information | |
|---|---|
| Concentration | 1 mg/mL |
| Purity | Contains approximately 90% NF-L and a small amount of NF-M. |
| Entrez Gene Number |
|
| Gene Symbol |
|
| UniProt Number |
|
| UniProt Summary | FUNCTION: Neurofilaments usually contain three intermediate filament proteins: L, M, and H which are involved in the maintenance of neuronal caliber. SIZE: 549 amino acids; 62072 Da 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. PTM: O-glycosylated (By similarity). 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). & 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. SIMILARITY: Belongs to the intermediate filament family. 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. |
| Product Usage Statements | |
|---|---|
| Usage Statement |
|
| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Maintain at -20°C to -70°C in undiluted aliquots for up to 6 months after date of receipt. Avoid repeated freeze/thaw cycles. |
| Packaging Information | |
|---|---|
| Material Size | 100 µg |