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The nuclear envelope (NE) is a specialized extension of the ER that contains numerous pore complexes interconnected with the nuclear lamina. The nuclear lamina composes the structural framework for the NE and serves as a chromatin anchor site, thus playing a major role in interphase nuclear organization. The major component of nuclear lamina are intermediate filament-like proteins called lamins. In mammalian somatic cells, there are three major lamins, A, B1, and C, and two minor lamins, B2 and A10. A-type lamins (A, A10, and C) are encoded by a single gene and are produced by alternative splicing, while B-type lamins (B1 and B2) are encoded by separate genes. B-type lamins are found in all nucleated somatic cells, while the expression of A-type lamins are developmentally regulated. Mice lacking lamin A show no overt abnormalities until postnatal development when perturbations in nuclear envelop structure correlate with the appearance of muscular dystrophy. In addition, lamin A is mutated in lipodystrophy, a disorder characterized by reduction in subcutaneous adipose tissue. Thus, lamin A and C may be important for nuclear envelope formation during postnatal cell differentiation.Immunofluorescence, Western Blotting
