Lamin A C Protein Size

Progerin is most often generated by a point mutation c1824t in the lmna gene that codes for lamin a and c.

Lamin a c protein size. Biogenesis of lamin a in normal cells and the failure to generate mature lamin a in hutchinson gilford progeria. Lamins are a class of intermediate filament proteins that form a matrix on the inner surface of the nuclear envelope. Progerin is a 614 amino acid protein involved in hutchinson gilford progeria syndrome. Lamin a mutation impairs interaction with nucleoporin nup155 and disrupts nucleocytoplasmic transport in atrial fibrillation.

These proteins are found in many different cell types in three different forms a b and c. Samples were fixed with 3 7 paraformaldehyde for 1 hour and stained for nuclear dna dapi filamentous actin tumor recognition antigen 1 81 and nuclear envelope protein lamin a. For staining cells were permeabilized with 0 1 triton x 100 for 10 min. Unlike lamin c lamin a is generated in a precursor form called prelamin a.

Lamins a and c are alternatively spliced versions of the lmna gene. Two isoforms lamins a and c can be created from this gene via alternative splicing. View protein in interpro ipr018039 if conserved ipr039008 if rod dom ipr042180 if rod dom coil1b ipr001322 lamin tail dom ipr036415 lamin tail dom sf. By protein lamin a c antibodies.

Lamin c 572 amino acids differs from lamin a after amino acid 566 containing an alternative 6 amino acid c terminal end vsgsrr. During apoptosis lamin a c is specifically cleaved into a large 41 50 kda and a small 28 kda fragment 3 4. Lmna also known as lamin a c is a protein that in humans is encoded by the lmna gene. View protein in smart sm01391 filament 1 hit.

This creates a high amount of homology between the isoforms. Lamin a c belongs to the lamin family of proteins. Goat serum 10 in phosphate buffered saline was used to block nonspecific binding for. Lamin a c contains several protein domains.

2000 j struct biol 129 313 23. This mutation activates a cryptic splice site and gives rise to a form of lamin a with a 50 amino acids internal deletion within the carboxyl terminal. Lamin a c mutation p arg399cys weakens the interaction between nuclear lamina lamin a c and the nuclear pore complex nup155 leading to the development of atrial fibrillation. Lamin a c antibodies.

Prelamin a and lamin c differ in structure only at the carboxyl terminus. View protein in pfam pf00038 filament 1 hit pf00932 ltd 1 hit. It lacks the region encoded by exon 10 resulting in the loss of an acidic and a polyhistidine domain. Lamin adel10 has a mw of 65 kda and a pi of 8 theoretical pi 8 58.

The lack of lamin a c can be as a novel marker for undifferentiated embryonic stem cells and lamin a c expression is as an early indicator of differentiation pmid.