| 别名 | LMNB1;LMN2;LMNB;Lamin-B1 |
| 基因名称 | LMNB1 |
| 蛋白名称 | Lamin-B1 |
| 推荐应用 | |
| 反应物种 | Human,Mouse,Rat |
| 存储缓冲液 | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Human Gene Link | http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=4001 |
| Human Swissprot No. | P20700 |
| Human Swissprot Link | http://www.uniprot.org/uniprotkb/P20700/entry |
| Mouse Gene ID | 16906 |
| Mouse Gene Link | http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=16906 |
| Mouse Swissprot No. | P14733 |
| Mouse Swissprot Link | http://www.uniprot.org/uniprot/P14733 |
| Rat Gene ID | 116685 |
| Rat Gene Link | http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=116685 |
| Rat Swissprot No. | P70615 |
| Rat Swissprot Link | http://www.uniprot.org/uniprot/P70615 |
| 稀释度 | IHC-P 1:200-1:1000,WB 1:1000-1:5000,IF-P/IF-F/IF-ICC 1:200-1:1000,ELISA 1:5000-1:20000,IP 1:50-1:200 |
| 参考分子量 | 68kDa |
| 预测分子量 | 68kDa |
| 运输及保存条件 | Biological ice bag transportation. Store at -20°C for at least 12 months(Do not lower than -25°C). Avoid freeze/thaw cycles |
| 宿主 | Rabbit |
| 同种型 | Monoclonal, rabbit, IgG, Kappa |
| 背景介绍 | lamin B1(LMNB1) Homo sapiens This gene encodes one of the two B-type lamin proteins and is a component of the nuclear lamina. A duplication of this gene is associated with autosomal dominant adult-onset leukodystrophy (ADLD). Alternative splicing results in multiple transcript variants. [provided by RefSeq, Dec 2015], |
| 功能 | disease: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.,function: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.,miscellaneous:The structural integrity of the lamina is strictly controlled by the cell cycle, as seen by the disintegration and formation of the nuclear envelope in prophase and telophase, respectively.,PTM: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.,similarity:Belongs to the intermediate filament family.,subunit:Interacts with lamin-associated polypeptides IA, IB and 2., |
| 纯化 | Protein A |
| Clonality | Monoclonal |
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