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描述 产品目录编号 AG231 品牌系列 Chemicon®
商名 - Chemicon
描述 Neurofilament 70 kDa, porcine 概述 Purified porcine axonal neurofilament 70 (low molecular weight, NF-L). 产品信息 演示 Liquid in 6M Urea, 10 mM Phosphate, pH 7.5. 应用 主要应用 - Positive Control
应用说明 Western blotting
Protein standard
ELISA
Radioimmunoassay
Optimal working dilution must be determined by the end user.生物信息 浓缩 1 mg/mL 纯度 Contains approximately 90% NF-L and a small amount of NF-M. 品种 Porcine Entrez基因编号 - NM_006158.2
基因符号 - NFL
- NEFL
- CMT1F
- NF-L
- NF68
- CMT2E
UniProt编号 - P07196
UniProt汇总 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.产品使用声明 使用声明 - 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.
储存和货运信息 存储条件 Maintain at -20°C to -70°C in undiluted aliquots for up to 6 months after date of receipt. Avoid repeated freeze/thaw cycles. 包装信息 数量 100 µg
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