货号：AG231

品牌：Merck Millipore

规格：100Ug

目录价：￥3758.00

市场价格：￥3194.30

会员价格：￥3006.40

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Neurofilament 70 kDa, porcine View All» Trade Name: Chemicon (Millipore) View All» Qty/Pk: 100 µg View All» Product Overview: Purified porcine axonal neurofilament 70 (low molecular weight, NF-L). View All» Key Applications: Positive Control View All» Application Notes: Western blotting Protein standard ELISA Radioimmunoassay Optimal working dilution must be determined by the end user. View All» Usage Statement: Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. View All» UniProt Summary: FUNCTION: SwissProt: P07196 # Neurofilaments usually contain three intermediate filament proteins: L, M, and H which are involved in the maintenance of neuronal caliber.SIZE: 543 amino acids; 61517 Da DOMAIN: SwissProt: P07196 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: SwissProt: P07196 # 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. View All» Species: Porcine View All» Brand Family: Chemicon View All» Presentation: Liquid in 6M Urea, 10 mM Phosphate, pH 7.5. View All» UniProt Number: P07196 View All» 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. View All» Gene Symbol:

• NEFL

• CMT1F

• NF-L

• NF68

• CMT2E

• NFL
  View All» Product Name: Neurofilament 70 kDa, porcine View All» Entrez Gene Number: NM_006158.2 View All» Concentration: 1 mg/mL View All» Purity: Contains approximately 90% NF-L and a small amount of NF-M. View All»
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