核膜标志物（Nuclear Envelope Marker）核纤层蛋白(Lamin) 是紧贴核内膜的一层厚度为20～50nm的纤维蛋白层或纤维网络。核纤层与细胞质骨架、核骨架连成一个整体，一般认为核纤层将核被膜和染色质提供了结构支架。有学者研究认为：lamin蛋白与细胞凋亡及衰老有关联，它包括：核纤层蛋白A、核纤层蛋白B、核纤层蛋白C几个不同亚型的蛋白。

背景资料

The nuclear lamina consists of a two-dimensional matrix of proteins located next to the inner nuclear membrane. The lamin family of proteins make up the matrix and are highly conserved in evolution. During mitosis, the lamina matrix is reversibly disassembled as the lamin proteins are phosphorylated. Lamin proteins are thought to be involved in nuclear stability, chromatin structure and gene expression. Vertebrate lamins consist of two types, A and B. This gene encodes one of the two B type proteins, B1. Alternative splicing results in transcript variants and a duplication of this gene is associated with autosomal dominant adult-onset leukodystrophy (ADLD). [provided by RefSeq, Oct 2010].

产品应用

应用	已检合格种属	预测种属	推荐稀释比例
WB	Mouse		1:500-2000

交叉反应

交叉反应: Mouse

靶标

基因名

LMNB1

蛋白名

Lamin-B1

亚基

Homodimer. Interacts with lamin-associated polypeptides IA, IB and 2.

亚细胞定位

Nucleus inner membrane; Lipid-anchor; Nucleoplasmic side.

翻译后修饰

B-type lamins undergo a series of modifications, such as farnesylation and phosphorylation. Increased phosphorylation of the lamins occurs before envelope disintegration and probably plays a role in regulating lamin associations.

疾病

Defects in LMNB1 are the cause of leukodystrophy demyelinating autosomal dominant adult-onset (ADLD) [MIM:169500]. ADLD is a slowly progressive and fatal demyelinating leukodystrophy, presenting in the fourth or fifth decade of life. Clinically characterized by early autonomic abnormalities, pyramidal and cerebellar dysfunction, and symmetric demyelination of the CNS. It differs from multiple sclerosis and other demyelinating disorders in that neuropathology shows preservation of oligodendroglia in the presence of subtotal demyelination and lack of astrogliosis.

相似性

Belongs to the intermediate filament family.

功能

Lamins are components of the nuclear lamina, a fibrous layer on the nucleoplasmic side of the inner nuclear membrane, which is thought to provide a framework for the nuclear envelope and may also interact with chromatin.

lamin B1 Rabbit pAb (一抗) | Bioss

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