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An absence of nuclear lamins in keratinocytes leads to ichthyosis, defective epidermal barrier function, and intrusion of nuclear membranes and endoplasmic reticulum into the nuclear chromatin.
Jung, Hea-Jin; Tatar, Angelica; Tu, Yiping; Nobumori, Chika; Yang, Shao H; Goulbourne, Chris N; Herrmann, Harald; Fong, Loren G; Young, Stephen G.
Afiliación
  • Jung HJ; Molecular Biology Institute, University of California, Los Angeles, California, USA.
  • Tatar A; Department of Medicine, University of California, Los Angeles, California, USA.
  • Tu Y; Department of Medicine, University of California, Los Angeles, California, USA.
  • Nobumori C; Department of Medicine, University of California, Los Angeles, California, USA.
  • Yang SH; Department of Medicine, University of California, Los Angeles, California, USA.
  • Goulbourne CN; Department of Medicine, University of California, Los Angeles, California, USA.
  • Herrmann H; German Cancer Research Center, Heidelberg, Germany.
  • Fong LG; Department of Medicine, University of California, Los Angeles, California, USA lfong@mednet.ucla.edu sgyoung@mednet.ucla.edu.
  • Young SG; Molecular Biology Institute, University of California, Los Angeles, California, USA Department of Medicine, University of California, Los Angeles, California, USA Department of Human Genetics, University of California, Los Angeles, California, USA lfong@mednet.ucla.edu sgyoung@mednet.ucla.edu.
Mol Cell Biol ; 34(24): 4534-44, 2014 Dec.
Article en En | MEDLINE | ID: mdl-25312645
B-type lamins (lamins B1 and B2) have been considered to be essential for many crucial functions in the cell nucleus (e.g., DNA replication and mitotic spindle formation). However, this view has been challenged by the observation that an absence of both B-type lamins in keratinocytes had no effect on cell proliferation or the development of skin and hair. The latter findings raised the possibility that the functions of B-type lamins are subserved by lamins A and C. To explore that idea, we created mice lacking all nuclear lamins in keratinocytes. Those mice developed ichthyosis and a skin barrier defect, which led to death from dehydration within a few days after birth. Microscopy of nuclear-lamin-deficient skin revealed hyperkeratosis and a disordered stratum corneum with an accumulation of neutral lipid droplets; however, BrdU incorporation into keratinocytes was normal. Skin grafting experiments confirmed the stratum corneum abnormalities and normal BrdU uptake. Interestingly, the absence of nuclear lamins in keratinocytes resulted in an interspersion of nuclear/endoplasmic reticulum membranes with the chromatin. Thus, a key function of the nuclear lamina is to serve as a "fence" and prevent the incursion of cytoplasmic organelles into the nuclear chromatin.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Piel / Queratinocitos / Laminas / Ictiosis Tipo de estudio: Prognostic_studies Límite: Animals Idioma: En Revista: Mol Cell Biol Año: 2014 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Estados Unidos
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Piel / Queratinocitos / Laminas / Ictiosis Tipo de estudio: Prognostic_studies Límite: Animals Idioma: En Revista: Mol Cell Biol Año: 2014 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Estados Unidos