Journal article
Investigating the purpose of prelamin A processing
Nucleus (Austin, Tex.), Vol.2(1), pp.4-9
01/01/2011
DOI: 10.4161/nucl.2.1.13723
PMCID: PMC3104803
PMID: 21647293
Abstract
Lmna
yields two major protein products in somatic cells, lamin C and prelamin A. Mature lamin A is produced from prelamin A by four posttranslational processing steps—farnesylation of a carboxyl-terminal cysteine, release of the last three amino acids of the protein, methylation of the farnesylcysteine, and the endoproteolytic release of the carboxyl-terminal 15 amino acids of the protein (including the farnesylcysteine methyl ester). Although the posttranslational processing of prelamin A has been conserved in vertebrate evolution, its physiologic significance remains unclear. Here we review recent studies in which we investigated prelamin A processing with
Lmna
knock-in mice that produce exclusively prelamin A (
Lmna
PLAO
), mature lamin A (
Lmna
LAO
) or nonfarnesylated prelamin A (
Lmna
nPLAO
). We found that the synthesis of lamin C is dispensable in laboratory mice, that the direct production of mature lamin A (completely bypassing all prelamin A processing) causes no discernable pathology in mice, and that exclusive production of nonfarnesylated prelamin A leads to cardiomyopathy.
Details
- Title: Subtitle
- Investigating the purpose of prelamin A processing
- Creators
- Brandon SJ Davies - University of California, Los AngelesCatherine Coffinier - University of California, Los AngelesShao H Yang - University of California, Los AngelesRichard H Barnes - University of California, Los AngelesHea-Jin Jung - University of California, Los AngelesStephen G Young - University of California, Los AngelesLoren G Fong - University of California, Los Angeles
- Resource Type
- Journal article
- Publication Details
- Nucleus (Austin, Tex.), Vol.2(1), pp.4-9
- DOI
- 10.4161/nucl.2.1.13723
- PMID
- 21647293
- PMCID
- PMC3104803
- NLM abbreviation
- Nucleus
- ISSN
- 1949-1034
- eISSN
- 1949-1042
- Publisher
- Landes Bioscience
- Language
- English
- Date published
- 01/01/2011
- Academic Unit
- Fraternal Order of Eagles Diabetes Research Center; Biochemistry and Molecular Biology
- Record Identifier
- 9984293076902771
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