A-Type lamins are nuclear proteins required for the structural and functional integrity of the nucleus. In mammals, type A proteins are represented by lamins A and C, which are different transcripts arising from the same gene LMNA. This polyclonal antibody is able to detect full-lenght prelamin A and human Lamin A.
Rabbit Anti Human Lamin A
Description
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 A and C are present in equal amounts in the lamina of mammals. Plays an important role in nuclear assembly, chromatin organization, nuclear membrane and telomere dynamics. Required for normal development of peripheral nervous system and skeletal muscle and for muscle satellite cell proliferation. Required for osteoblastogenesis and bone formation. Also prevents fat infiltration of muscle and bone marrow, helping to maintain the volume and strength of skeletal muscle and bone .
Mutations in lamin A produce a range of diseases that have collectively been referred to as `laminopathies’ . Two progeroid (premature aging) syndromes, Hutchinson-Gilford progeria (HGPS) and restrictive dermopathy (RD), are laminopathies that arise through defects in maturation of the lamin A precursor, prelamin A. Mandibuloacral dysplasia (MAD), which can be considered a milder form of RD, also results in accumulation of the lamin A precursor. By contrast, the majority of laminopathies are due to point mutations in the A/C lamins; these exhibit a multitude of phenotypes depending on the site of the mutation .
Additional information
| Size | 200 µL |
|---|---|
| Application | Clinical diagnostics| Life Sciences |
| Product type | Reagents for research |
| Reagent type | Antibody |
| Category | Laminopathies |
| Subcategory | Polyclonal anti human Lamin A and Prelamin A |
| Antibody type | Primary antibody |
| Immunogen | Full-lenght recombinant Lamin A |
Technical information
| Size | 200 µL |
|---|---|
| Purity | Purified by affinity Chromatography |
| Form | Liquid. Supplied in 100mM sodium citrate, 50mM Tris and 0.05% v/v glycerol. Neutral pH. |
| Shipping/storage conditions | Shipped at 2-8°C. When stored at +4°C the antibody is stable for 18 months. For extended storage up to 24 months, the solution may be frozen at -20°C in working aliquots. Avoid repested freezing and thawing cycles. |
| Usage | For Laboratory Research Use Only |
| Source | Rabbit |
| Specificity | Antibody binds with high affinity down to 50 ng of Lamin A and Prelamin A in cells lines and in patient-derived specimens |
| React with | Human Prelamin A and Lamin A. |
| Recommended dilutions | Recommended starting dilutions can vary lot-to-lot. Consult the suggested starting dilutions on the top of the page for lot specific values. |
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References
| Nuclear Nox4 interaction with prelamin A is associated with nuclear redox control of stem cell aging | PUBMED ID: 30362963 |
| Samp1 Mislocalization in Emery-Dreifuss Muscular Dystrophy | PUBMED ID: 30326651 |
| The relevance of prelamin A and RAD51 as molecular biomarkers in cervical cancer | PUBMED ID: 29212225 |
| Statins and Histone Deacetylase Inhibitors Affect Lamin A/C - Histone Deacetylase 2 Interaction in Human Cells | PUBMED ID: 30766871 |
