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LMNA / Lamin A/C Antibody (clone 133A2)
Mouse Monoclonal Antibody
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application 
| WB, IHC-P, ICC, IHC-Fr, Flo |
|---|---|
| Primary Accession | P02545 |
| Other Accession | 4000 |
| Reactivity | Human, Mouse, Rat, Bovine, Dog |
| Host | Mouse |
| Clonality | Monoclonal |
| Isotype | IgG3 |
| Clone Names | 133A2 |
| Calculated MW | 74139 Da |
| Dilution | Flo (1:100 - 1:200), IHC-Fr (1:100 - 1:200), IHC-P (10 µg/ml), WB (1:100 - 1:1000), |
| Gene ID | 4000 |
|---|---|
| Other Names | LMNA, 70 kDa lamin, CDCD1, CMD1A, EMD2, FPLD, IDC, FPL, FPLD2, Lamin A/C, Lamin, LFP, LMNC, LMN1, LMNL1, HGPS, Prelamin-A/C, PRO1, Lamin A, Lamin A/C-like 1, LGMD1B, CDDC, CMT2B1, LDP1 |
| Target/Specificity | Recognizes an epitope located between residues 598-611 of lamin A and therefore reacts exclusively with lamin A. |
| Reconstitution & Storage | PBS containing 0.09% sodium azide. Short term 4°C, long term aliquot and store at -20°C, avoid freeze thaw cycles. Store undiluted. |
| Precautions | LMNA / Lamin A/C Antibody (clone 133A2) is for research use only and not for use in diagnostic or therapeutic procedures. |
| Name | LMNA |
|---|---|
| Synonyms | LMN1 |
| Function | [Lamin-A/C]: Lamins are intermediate filament proteins that assemble into a filamentous meshwork, and which constitute the major components of the nuclear lamina, a fibrous layer on the nucleoplasmic side of the inner nuclear membrane (PubMed:2344612, PubMed:2188730, PubMed:24741066, PubMed:10080180, PubMed:10580070, PubMed:10587585, PubMed:10814726, PubMed:11799477, PubMed:12075506, PubMed:12927431, PubMed:15317753, PubMed:18551513, PubMed:18611980, PubMed:22431096, PubMed:23666920, PubMed:31434876, PubMed:31548606, PubMed:37788673, PubMed:37832547). Lamins provide a framework for the nuclear envelope, bridging the nuclear envelope and chromatin, thereby playing an important role in nuclear assembly, chromatin organization, nuclear membrane and telomere dynamics (PubMed:24741066, PubMed:10080180, PubMed:10580070, PubMed:10587585, PubMed:10814726, PubMed:11799477, PubMed:12075506, PubMed:12927431, PubMed:15317753, PubMed:18551513, PubMed:18611980, PubMed:22431096, PubMed:23666920, PubMed:31548606, PubMed:37788673, PubMed:37832547). Lamin A and C also regulate matrix stiffness by conferring nuclear mechanical properties (PubMed:25127216, PubMed:23990565). 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 (PubMed:2344612, PubMed:2188730). Lamin A and C are present in equal amounts in the lamina of mammals (PubMed:10080180, PubMed:10580070, PubMed:10587585, PubMed:10814726, PubMed:11799477, PubMed:12075506, PubMed:12927431, PubMed:15317753, PubMed:18551513, PubMed:18611980, PubMed:22431096, PubMed:23666920, PubMed:31548606). Also invoved in DNA repair: recruited by DNA repair proteins XRCC4 and IFFO1 to the DNA double-strand breaks (DSBs) to prevent chromosome translocation by immobilizing broken DNA ends (PubMed:31548606). Required for normal development of peripheral nervous system and skeletal muscle and for muscle satellite cell proliferation (PubMed:10080180, PubMed:10814726, PubMed:11799477, PubMed:18551513, PubMed:22431096). Required for osteoblastogenesis and bone formation (PubMed:12075506, PubMed:15317753, PubMed:18611980). Also prevents fat infiltration of muscle and bone marrow, helping to maintain the volume and strength of skeletal muscle and bone (PubMed:10587585). Required for cardiac homeostasis (PubMed:10580070, PubMed:12927431, PubMed:23666920, PubMed:18611980). |
| Cellular Location | Nucleus lamina. Nucleus envelope. Nucleus, nucleoplasm. Nucleus matrix. Note=Farnesylation of prelamin-A/C facilitates nuclear envelope targeting and subsequent cleavage by ZMPSTE24/FACE1 to remove the farnesyl group produces mature lamin-A/C, which can then be inserted into the nuclear lamina (PubMed:15317753) EMD is required for proper localization of non-farnesylated prelamin- A/C (PubMed:19323649). Also localizes to the micronuclear envelope in response to response to genome instability (PubMed:37788673) |
| Tissue Location | In the arteries, prelamin-A/C accumulation is not observed in young healthy vessels but is prevalent in medial vascular smooth muscle cells (VSMCs) from aged individuals and in atherosclerotic lesions, where it often colocalizes with senescent and degenerate VSMCs. Prelamin-A/C expression increases with age and disease. In normal aging, the accumulation of prelamin-A/C is caused in part by the down-regulation of ZMPSTE24/FACE1 in response to oxidative stress. |
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Provided below are standard protocols that you may find useful for product applications.
Background
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.
References
McKeon F.D.,et al.Nature 319:463-468(1986).
Fisher D.Z.,et al.Proc. Natl. Acad. Sci. U.S.A. 83:6450-6454(1986).
Sylvius N.,et al.J. Med. Genet. 42:639-647(2005).
Csoka A.B.,et al.Submitted (JUL-2003) to the EMBL/GenBank/DDBJ databases.
Ota T.,et al.Nat. Genet. 36:40-45(2004).
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