Information about Nuclear Lamina

The nuclear lamina is a dense, ~ 30 to 100 nanometers thick, fibrillar network composed of intermediate filaments made of lamin that lines the inner surface of the nuclear envelope in animal cells. At the onset of mitosis, a certain cyclin-dependent kinase complex (Cdk1 and cyclin B in mammals) phosphorylates the lamins, causing them to undergo a conformational change that triggers the disassembly of the nuclear envelope. After the chromosomes have migrated to each pole, the lamins are dephosphorylated, allowing them to begin to reform the nuclear lamina. Initially the lamina reassembles directly on the outer surface of the condensed chromosomes by binding to proteins which associate the condensed DNA, thus excluding all cytoplasmic proteins from the newly forming intranuclear space. The lamins also bind to lamin receptor proteins embedded in the membrane of the endoplasmic reticulum (the ER membrane is continuous with the nuclear envelope). This allows the nuclear envelope to reform around each of the chromosomes. As the chromosomes unwind, their individual coatings of ER membrane merge with one another to form a single nuclear envelope supported by the nuclear lamina. Even during interphase the lamins maintain interactions with DNA binding proteins or DNA itself, especially with heterochromatin which tends to cluster at the surface of the nuclear lamina.

Associated Pathologies

Main article: Laminopathies
Defects in the genes encoding for nuclear lamin (such as LMNA and LMNB1) have been implicated in a variety of diseases such as:

Further reading

  • Goldman et al., "Nuclear lamins: building blocks of nuclear architecture", Genes and Development 16:543-547, 2002. A good introductory article.

External links

Intermediate filaments (IFs) are cytoskeletal structures formed by members of a family of related proteins. Intermediate filaments have a diameter between that of actin (microfilaments) and microtubules.
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Lamins are fibrous proteins having structural function in the cell nucleus.

In metazoan cells there are A and B type lamins which differ in their length and pI. Human cells have three differentially regulated genes.
  • B-type lamins are present in every cell.

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The nuclear envelope (also known as the perinuclear envelope, nuclear membrane, nucleolemma or karyotheca) is the double membrane of the nucleus that encloses genetic material in eukaryotic cells.
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A cyclin-dependent kinase complex (abbreviated cdkc, also called cyclin-CDK) is a protein complex formed by the association of cyclin with cyclin-dependent kinase. These complexes are able to phosphorylate other compounds under consumption of ATP, and are thereby involved in
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Figure 1: A representation of a condensed eukaryotic chromosome, as seen during cell division.]] A chromosome is a single large macromolecule of DNA, and constitutes a physically organized form of DNA in a cell.
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Definition

Interphase is a phase of the cell cycle, defined only by the absence of cell division. During interphase, the cell obtains nutrients, and duplicates its chromatids. Most eukaryotic cells spend most of their time in interphase.
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Heterochromatin is a tightly packed form of DNA. Its major characteristic is that transcription is limited.

Structure

Chromatin is found in two varieties: euchromatin and heterochromatin.
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Laminopathies are a group of rare genetic disorders caused by mutations in genes encoding proteins of the nuclear lamina. They are included in the more generic term nuclear envelopathies that was coined in 2000 for diseases associated with defects of the nuclear envelope.
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MeSH D020389 Emery-Dreifuss muscular dystrophy is a condition that chiefly affects muscles used for movement (skeletal muscles) and heart (cardiac) muscle. Among the earliest features of this disorder are joint deformities called contractures, which restrict the movement of
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Progeria
Classification & external resources

ICD-10 E 34.8
ICD-9 259.8

OMIM 176670
DiseasesDB 10704

eMedicine derm/731  
MeSH D011371 The term progeria narrowly refers to Hutchinson-Gilford Progeria syndrome
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Medical Subject Headings (MeSH) is a huge controlled vocabulary (or metadata system) for the purpose of indexing journal articles and books in the life sciences. Created and updated by the United States National Library of Medicine (NLM), it is used by the MEDLINE/PubMed
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