lamin-A
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Function
- lamins are components of the nuclear lamina (see lamin)
- lamin A & lamin C filaments cross-link into an orthogonal lattice (nuclear lamina), which is attached via lamin B to the inner nuclear membrane through interactions with a lamin B receptor
- increased phosphorylation of the lamins occurs before nuclear envelope disintegration & probably plays a role in regulating lamin associations
- the C-terminal 18 residues are removed by proteolytic cleavage of lamin A; cleavage requires prior farnesylation
- proteolytically processed Lamin A interacts with NARF
Structure
- belongs to the intermediate filament family
- a 2-3 kD fragment is cleaved from the C-terminus
- farnesylated
Compartment
Pathology
- truncation of Lamin A associated with:[3] Hutchinson-Gilford Progeria syndrome
- unprocessed prelamin A & truncated lamin A act dominant negatively to perturb cell cycle checkpoint control, DNA damage response & DNA repair, resulting in genomic instability[3]
More general terms
References
- ↑ Holtz D et al The CaaX motif of lamin A functions in conjunction with the nuclear localization signal to target assembly to the nuclear envelope. Cell 59:969 1989 PMID: https://www.ncbi.nlm.nih.gov/pubmed/2557160
- ↑ Glomset JA et al Prenyl proteins in eukaryotic cells: a new type of membrane anchor. TIBS 15(April):139 1990 PMID: https://www.ncbi.nlm.nih.gov/pubmed/2187294
- ↑ 3.0 3.1 3.2 Liu B et al Genomic instability in laminopathy-based premature aging. Nat Med. 2005 Jul;11(7):780-5. Epub 2005 Jun 26 PMID: https://www.ncbi.nlm.nih.gov/pubmed/15980864
- ↑ UniProt http://www.uniprot.org/uniprot/P02545.html