Dermal fibroblasts in Hutchinson-Gilford progeria syndrome with the lamin A G608G mutation have dysmorphic nuclei and are hypersensitive to heat stress.

Paradisi, Mauro; McClintock, Dayle; Boguslavsky, Revekka L; Pedicelli, Christina; Worman, Howard J; Djabali, Karima

Paradisi, Mauro; McClintock, Dayle; Boguslavsky, Revekka L; Pedicelli, Christina; Worman, Howard J; Djabali, Karima.

Affiliation

Paradisi M; VII Divisione, Dermatologia Pediatrica, Istituto Dermopatico Dell'Immacolata IRCCS, Rome, Italy. m.paradisi@idi.it

BMC Cell Biol ; 6: 27, 2005 Jun 27.

Article in En | MEDLINE | ID: mdl-15982412

Subject(s)

Fibroblasts/pathology; Heat Stress Disorders/genetics; Lamin Type A/genetics; Point Mutation; Progeria/genetics; Cell Nucleus/chemistry; Cell Nucleus/pathology; Cell Nucleus/ultrastructure; Cells, Cultured; Child, Preschool; Chromatin; Female; Fibroblasts/ultrastructure; Humans; Lamin Type A/analysis; Lamins/analysis; Membrane Proteins/analysis; Nuclear Envelope/pathology; Nuclear Proteins; Progeria/pathology; Sequence Deletion; Skin/pathology; Thymopoietins/analysis

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Full text: 1 Database: MEDLINE Main subject: Progeria / Point Mutation / Heat Stress Disorders / Lamin Type A / Fibroblasts Limits: Child, preschool / Female / Humans Language: En Year: 2005 Type: Article

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