LMNA

The following LMNA reagents supplied by CUSABIO are manufactured under a strict quality control system. Multiple applications have been validated and solid technical support is offered.

LMNA Antibodies

LMNA Antibodies for Homo sapiens (Human)

LMNA Proteins

LMNA Proteins for Gallus gallus (Chicken)

LMNA Proteins for Rattus norvegicus (Rat)

LMNA Proteins for Mus musculus (Mouse)

LMNA Proteins for Homo sapiens (Human)

LMNA Proteins for Xenopus laevis (African clawed frog)

LMNA Proteins for Sus scrofa (Pig)

LMNA ELISA Kit

LMNA ELISA Kit for Homo sapiens (Human)

LMNA Background

The human LMNA gene encodes nuclear lamins A and lamins C [1], collectively called A-type lamins, major protein constituents of the mammalian nuclear lamina, a complex structure that acts as a scaffold for protein complexes that regulate nuclear structure and functions [2]. Prelamin A and lamin C are identical through their first 566 amino acids, but then their sequences diverge [1]. Lamins A and C are expressed at very low levels early in embryonic development but are expressed at high levels in most differentiated cells [3-5]. Pre–lamin A undergoes a series of posttranslational modifications that culminate with the cleavage of its farnesylated 15 C-terminal residues to generate mature lamin A [6]. Lamin C does not have a CAAX motif and is never farnesylated in the first place [1]. Besides the well-established role in maintaining the mechanical stability of the nucleus, increasing evidence has shown that A-type lamins and associated nuclear envelope (NE) proteins are scaffolds for proteins that regulate DNA synthesis, responses to DNA damage, chromatin organization, gene transcription, cell cycle progression, cell differentiation, and cell migration [6][7]. Nearly all known LMNA-associated human laminopathies are caused by single "missense" mutations in A-type lamin proteins [8].

[1] Lin F, Worman HJ. Structural organization of the human gene encoding nuclear lamin A and nuclear lamin C [J]. J Biol Chem. 1993 Aug 5; 268(22):16321-6.
[2] Vicente Andrés, José M. González. Role of A-type lamins in signaling, transcription, and chromatin organization [J]. J Cell Biol (2009) 187 (7): 945-957.
[3] Eckersley-Maslin MA, Bergmann JH, et al. Lamin A/C is expressed in pluripotent mouse embryonic stem cells [J]. Nucleus 2013, 4:53-60.
[4] Constantinescu D, Gray HL, et al. Lamin A/C expression is a marker of mouse and human embryonic stem cell differentiation [J]. Stem Cells 2006, 24:177-185.
[5] Rober RA, Weber K, et al. Differential timing of nuclear lamin A/C expression in the various organs of the mouse embryo and the young animal: a developmental study [J]. Development 1989, 105, 365-378.
[6] Broers J.L., Ramaekers F.C., et al. Nuclear lamins: laminopathies and their role in premature ageing [J]. Physiol. Rev. 2006, 86:967-1008.
[7] Verstraeten V.L., Broers J.L., et al. The nuclear envelope, a key structure in cellular integrity and gene expression [J]. Curr. Med. Chem. 2007, 14:1231-1248.
[8] Worman HJ, Fong LG, et al. Laminopathies and the long strange trip from basic cell biology to therapy [J]. J Clin Invest 2009, 119, 1825-1836.

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