InnateDB: Systems Biology of the Innate Immune Response

Mus musculus Protein: Lmna

Summary

InnateDB Protein

IDBP-401142.4

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

Lmna

Protein Name

lamin A

Synonyms

Dhe;

Species

Mus musculus

Ensembl Protein

ENSMUSP00000113093

InnateDB Gene

IDBG-160992 (Lmna)

Protein Structure

UniProt Annotation

Function

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. Isoform C2 may have a role in determining the organization of nuclear and chromosomal structures during spermatogenesis. {ECO:0000269PubMed:10579712, ECO:0000269PubMed:11799477, ECO:0000269PubMed:19124654, ECO:0000269PubMed:21547077, ECO:0000269PubMed:21982926, ECO:0000269PubMed:23535822}.Prelamin-A/C can accelerate smooth muscle cell senescence. It acts to disrupt mitosis and induce DNA damage in vascular smooth muscle cells (VSMCs), leading to mitotic failure, genomic instability, and premature senescence (By similarity). {ECO:0000250}.

Subcellular Localization

Nucleus. Nucleus envelope. Nucleus lamina {ECO:0000250}. Nucleus, nucleoplasm {ECO:0000250}. Note=Farnesylation of prelamin-A/C facilitates nuclear envelope targeting and subsequent cleaveage by ZMPSTE24/FACE1 to remove the farnesyl group produces mature lamin-A/C, which can then be inserted into the nuclear lamina. EMD is required for proper localization of non-farnesylated prelamin-A/C (By similarity). {ECO:0000250}.

Disease Associations

Tissue Specificity

Expressed in liver and in bone marrow (at protein level). Isoform C2 is specifically expressed in germ cells. {ECO:0000269PubMed:10579712, ECO:0000269PubMed:21547077}.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 69 experimentally validated interaction(s) in this database.
They are also associated with 71 interaction(s) predicted by orthology.

Experimentally validated
Total	69 [view]
Protein-Protein	68 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	1 [view]
RNA-RNA	0
DNA-RNA	0

Predicted by orthology
Total	71 [view]

Gene Ontology

Molecular Function

Accession	GO Term
GO:0005198	structural molecule activity
GO:0005515	protein binding

Biological Process

GO:0006997	nucleus organization
GO:0006998	nuclear envelope organization
GO:0007517	muscle organ development
GO:0030010	establishment of cell polarity
GO:0030334	regulation of cell migration
GO:0030951	establishment or maintenance of microtubule cytoskeleton polarity
GO:0034504	protein localization to nucleus
GO:0035105	sterol regulatory element binding protein import into nucleus
GO:0055015	ventricular cardiac muscle cell development
GO:0071456	cellular response to hypoxia
GO:0090201	negative regulation of release of cytochrome c from mitochondria
GO:0090343	positive regulation of cell aging
GO:1900180	regulation of protein localization to nucleus
GO:2001237	negative regulation of extrinsic apoptotic signaling pathway

Cellular Component

GO:0005634	nucleus
GO:0005635	nuclear envelope
GO:0005638	lamin filament
GO:0005654	nucleoplasm
GO:0005882	intermediate filament
GO:0048471	perinuclear region of cytoplasm