Lamins function as skeletal components tightly associated with the inner nuclear membrane. Originally the proteins of the nuclear cytoskeleton were named Lamin A, B and C, from top to bottom as visualized on SDS-PAGE gels. Subsequently, it was found that Lamins A and C were coded for by a single gene, while the Lamin B band may contain two proteins encoded by two genes now called Lamin B1 and Lamin B2. Lamin A has a mass of about 74kDa while Lamin C is 65kDa. The Lamin A protein includes 98 amino acids missing from Lamin C, while Lamin C has a C-terminal 6 amino acid peptide not present in Lamin A. Apart from these regions Lamin A and C are identical so that antibodies raised against either protein are likely to cross-react with the other, as is the case with this monoclonal.
The activity of this kinase increases during cell division and is responsible for the breakdown of the nuclear lamina. Mutations in the LMNA gene are associated with several serious human diseases, including Emery-Dreifuss muscular dystrophy, familial partial lipodystrophy, limb girdle muscular dystrophy, dilated cardiomyopathy, Charcot-Marie-Tooth disease type 2B1, and Hutchinson-Gilford progeria syndrome. This family of diseases belongs to a larger group which are often referred to as Laminopathies, though some laminopathies are associated in defects in Lamin B1, B2 or one or other of the numerous nuclear lamina binding proteins. A truncated version of lamin A, commonly known as progerin, causes Hutchinson-Gilford progeria syndrome, a form of premature aging.
- Product Reviews
- Product Publications
If you've used this product in a publication, let us know. Email email@example.com, with the publication details and you could be eligible for an Amazon gift card.
- Related Products
Name Catalog # Size ATPase Na+/K+ transporting alpha 1 MO25023 50 ul Fibrillarin CH22129 100 ul Fibrillarin MO22169 100 µl Fibrillarin RA22147 100 ul Fibrillarin/Nop1p- Nucleolar Marker MO22108 500 ul FluoGreen Tracer SF41000 100 ul GFP GT22101 100 ul Green Fluorescent Protein CH22124 100 ul of 1 mg/ml Green Fluorescent Protein MO22184 100 ul Green Fluorescent Protein (GFP) MO22190 100 ul Green Fluorescent Protein (GFP) RA22134 100 ul of 1 mg/ml High-mobility group protein B1/HMGP1 MO22134 100 ul Lamin A/C CH22136 100 ul mCherry GT22108 100 ul mCherry MO22192 100 ul mCherry - Mouse MO22140 100 ul mCherry-Chicken CH22115 100 ul mCherry-Rabbit RA22117 100 ul MiTF (Microphthalmia-Associated Transcription Factor) MO47034 0.5 ml Panspecific Nuclear Pore Complex Marker MO22107 500 ul S-Tag/S15 MO22203 100 ul S-Tag/S15 MO22206 100 ul TAF15 MO22114 100 ul Tar DNA Binding Protein 43 (TDP43) MO22117 100 ul