Nuclear Envelope Protein Lamin

Transcription factors that bind to the lamina include the retinoblastoma transcriptional regulator rb germ cell less gcl sterol response element binding protein srebp1.

Nuclear envelope protein lamin. A mouse model for this rare disease has been created by. We used these proteins together with ce lamin and nucleoporins to determine the timing of nuclear envelope disassembly in c. We discovered unexpected differences in the timing of nuclear envelope breakdown between c. Lamin b1 is a scaffolding supporting component of the nuclear envelope which is the structure that surrounds the nucleus in cells.

The nuclear lamina is composed mainly of type v intermediate filament proteins termed lamins which are classified into a and. The nuclear envelope contains nuclear pore complexes npcs to facilitate exchange of molecules with the cytoplasm 5 and in metazoans the inm is connected to a filamentous network termed the nuclear lamina 6 8. Knockout tested rabbit polyclonal lamin b1 antibody nuclear envelope marker. Genetic studies have now shown that people with mutations in either lamins a c or emerin a nuclear membrane protein develop emery dreifuss muscular dystrophy.

Specifically this protein is located in the nuclear lamina a mesh like layer of intermediate filaments and other proteins that is attached to the inner membrane of the nuclear envelope. Lamin a is also farnesylated but it is unique among farnesylated human proteins 90. Lamins and other nuclear envelope proteins organize nuclear architecture through structural attachments that vary dynamically during the cell cycle and cell differentiation. Elegans and vertebrates or drosophila and also between early 2 to 24 cell and later 30 cell stages of.

Following modifications by ftase rce1 and icmt prelamin a undergoes a. Been suggested that nuclear envelope ne proteins a hallmark of eukaryotic cells participate in the mechano transduction networks. Nuclear envelope proteins that are bound to the lamina include nesprin emerin lamina associated proteins 1 and 2 lap1 and lap2 the lamin b receptor lbr and man1. Our previous proteomic analysis revealed that lamin a c one kind of ne protein is mechano responsive and may contribute to the shear stress induced proliferation and migration of vsmcs 12.

Mutations in lamin genes can result in several genetic laminopathies which may be life threatening. Validated in wb ihc icc if and tested in mouse rat human indian muntjac. Lamin proteins are involved in the disassembling and reforming of the nuclear envelope during mitosis the positioning of nuclear pores and programmed cell death. As more and more unrelated diseases were linked to lamins and other nuclear envelope proteins nuclear structure and disease became an increasingly prominent research focus.

When mutations in nuclear lamins were first identified in skeletal and cardiac muscle diseases the molecular events underlying pathogenesis were mere points of speculation.