低分子量神经丝蛋白重组兔单抗

神经生物学相关蛋白（Neurobiology）
低分子量神经丝蛋白,简称NF-L,分子量为68kDa，NF-L的聚集与神经退行性疾病的发病机理相关，如运动神经元的降解等。
神经纤丝蛋白的功能是提供弹性使神经纤维易于伸展和防止断裂。
神经丝是中间纤维的一种重要类型又称神经微丝蛋白，特异地在神经细胞内表达，并在轴突内相互平行排列成束.　哺乳动物的神经丝由3种蛋白组成：
低分子量神经丝蛋白，简称NF-L；分子量为68kDa；
中分子量神经丝蛋白，简称NF-M；分子量为160kDa；
高分子量神经丝蛋白，简称NF-H，分子量为200 kDa。

背景资料

Neurofilament light polypeptide also called NF-L; Neurofilament triplet L protein; 68 kDa neurofilament protein. Neurofilaments usually contain three intermediate filament proteins: L, M, and H which are involved in the maintenance of neuronal caliber. 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. 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. Belongs to the intermediate filament family.
Involved in the maintenance of neuronal caliber, neurofilaments are the intermediate filament proteins found specifically in neurons, and are composed predominantly of three major proteins called NF-L, NF-M and NF-H. Like most other intermediate filament proteins (IFPs), the expression of the different neuronal IFPs is both tissue-specific and developmentally regulated. NF-L is the light or low molecular weight microfilament subunit and runs on SDS-PAGE gels at approximately 70 kDa. Neurofilament 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-H is the heavy or high molecular weight microfilament subunit and runs on SDS-PAGE gels in the range 180-220 kDa, with some variation in different species.

产品应用

应用	已检合格种属	预测种属	推荐稀释比例
WB	Human, Mouse	Rat	1:500-5000
IHC-P	Human, Mouse, Rat		1:100-500
IHC-F	Human, Mouse, Rat		1:100-500
IF	Human, Mouse, Rat		1:100-500

交叉反应

交叉反应: Human, Mouse, Rat

靶标

基因名

NEFL

蛋白名

Neurofilament light polypeptide

亚基

Interacts with ARHGEF28. Interacts with TRIM2.

翻译后修饰

O-glycosylated.
Phosphorylated in the head and rod regions by the PKC kinase PKN1, leading to the inhibition of polymerization.
Ubiquitinated in the presence of TRIM2 and UBE2D1.

疾病

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.

相似性

Belongs to the intermediate filament family.

功能

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

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