Abstract：
:Mutations in the LMNA gene, which encodes nuclear lamins A and C by alternative splicing, can give rise to Emery-Dreifuss muscular dystrophy. The mechanism by which lamins A and C separately contribute to this molecular phenotype is unknown. To address this question we examined ten LMNA mutations exogenously expressed as lamins A and C in COS-7 cells. Eight of the mutations when expressed in lamin A, exhibited a range of nuclear mislocalisation patterns. However, two mutations (T150P and delQ355) almost completely relocated exogenous lamin A from the nuclear envelope to the cytoplasm, disrupted nuclear envelope reassembly following cell division and altered the protein composition of the mid-body. In contrast, exogenously expressed DsRed2-tagged mutant lamin C constructs were only inserted into the nuclear lamina if co-expressed with any EGFP-tagged lamin A construct, except with one carrying the T150P mutation. The T150P, R527P and L530P mutations reduced the ability of lamin A, but not lamin C from binding to emerin. These data identify specific functional roles for the emerin-lamin C- and emerin-lamin A- containing protein complexes and is the first report to suggest that the A-type lamin mutations may be differentially dysfunctional for the same LMNA mutation.
journal_name
Eur J Cell Bioljournal_title
European journal of cell biologyauthors
Motsch I,Kaluarachchi M,Emerson LJ,Brown CA,Brown SC,Dabauvalle MC,Ellis JAdoi
10.1016/j.ejcb.2005.04.004subject
Has Abstractpub_date
2005-09-01 00:00:00pages
765-81issue
9eissn
0171-9335issn
1618-1298pii
S0171-9335(05)00087-7journal_volume
84pub_type
杂志文章abstract：:Centaurin alpha-1 is a high-affinity PtdIns(3,4,5)P3-binding protein enriched in brain. Sequence analysis indicates centaurin alpha-1 contains two pleckstrin homology domains, ankyrin repeats and an Arf GAP homology domain, placing it in the AZAP family of phosphoinositide-regulated Arf GAPs. Other members of this fam...
journal_title：European journal of cell biology
pub_type： 杂志文章
doi：10.1078/0171-9335-00416
更新日期：2004-10-01 00:00:00
abstract：:The exhaustion of metabolically poisoned muscle fibres is accompanied by a one hundred-fold increase in potassium conductance. Under these conditions a normal membrane capacity was measured. Extracellular markers (lanthanum, ferritin) were traced in the transverse tubular system but not in the myoplasm or the sarcopla...
journal_title：European journal of cell biology
pub_type： 杂志文章
doi：
更新日期：1980-04-01 00:00:00
abstract：:Yeast dynamin, Vacuolar Protein Sorting 1 (Vps1), has been implicated in recycling traffic from the endosome to the trans-Golgi network (TGN). Previous research showed a genetic interaction of Vps1 with all components of the GARP tethering complex, which anchors vesicles at the late Golgi membrane. We used the yeast t...
journal_title：European journal of cell biology
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doi：10.1016/j.ejcb.2017.04.004
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journal_title：European journal of cell biology
pub_type： 杂志文章
doi：10.1016/j.ejcb.2014.10.007
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abstract：:Cytochrome b5 has been visualized by an immunoferritin labeling in a Golgi preparation. When the Golgi apparatus was first disrupted before labeling, 60% of the profiles were clearly labeled with ferritin. This proportion of labeled vesicles was too high to be explained by contamination from other membranes. When the ...
journal_title：European journal of cell biology
pub_type： 杂志文章
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abstract：:Changes in the surface morphology of regenerating rabbit, rat and frog corneal endothelial cells in vivo have been investigated by scanning electron microscopy. In adult tissue these cells do not normally divide unless given a stimulus, such as injury. Surfaces of quiescent rabbit and rat cells are devoid of microvill...
journal_title：European journal of cell biology
pub_type： 杂志文章
doi：
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journal_title：European journal of cell biology
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journal_title：European journal of cell biology
pub_type： 杂志文章
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journal_title：European journal of cell biology
pub_type： 杂志文章
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journal_title：European journal of cell biology
pub_type： 杂志文章
doi：
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journal_title：European journal of cell biology
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journal_title：European journal of cell biology
pub_type： 杂志文章
doi：10.1016/j.ejcb.2017.02.004
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journal_title：European journal of cell biology
pub_type： 杂志文章
doi：
更新日期：1981-02-01 00:00:00
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journal_title：European journal of cell biology
pub_type： 杂志文章
doi：
更新日期：1981-08-01 00:00:00
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journal_title：European journal of cell biology
pub_type： 杂志文章
doi：
更新日期：1994-06-01 00:00:00
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journal_title：European journal of cell biology
pub_type： 杂志文章
doi：
更新日期：1992-06-01 00:00:00
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journal_title：European journal of cell biology
pub_type： 杂志文章
doi：
更新日期：1982-02-01 00:00:00
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journal_title：European journal of cell biology
pub_type： 杂志文章
doi：
更新日期：1991-04-01 00:00:00
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journal_title：European journal of cell biology
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doi：
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journal_title：European journal of cell biology
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doi：
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journal_title：European journal of cell biology
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doi：
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journal_title：European journal of cell biology
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doi：
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journal_title：European journal of cell biology
pub_type： 杂志文章
doi：
更新日期：1988-12-01 00:00:00
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journal_title：European journal of cell biology
pub_type： 杂志文章,评审
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更新日期：2012-01-01 00:00:00
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journal_title：European journal of cell biology
pub_type： 杂志文章
doi：
更新日期：1982-02-01 00:00:00
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journal_title：European journal of cell biology
pub_type： 杂志文章
doi：10.1078/0171-9335-00136
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journal_title：European journal of cell biology
pub_type： 杂志文章
doi：
更新日期：1985-05-01 00:00:00
