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Lamin B1 Regulation.pdf

Nmezi_Bruce_Dissertation_August2019.pdf
Preview of Lamin B1 Regulation
🔗 Source: d-scholarship.pitt.edu
📊 Size: 3.62 MB
👤 Author: Fudrow, John P
⬇️ Downloads: 169

Summary

The nuclear lamina, composed of lamins, maintains nuclear shape and function, and perturbations lead to laminopathies, including autosomal dominant leukodystrophy (ADLD), caused by lamin B1 overexpression. Studies suggest a novel concentric organization model of the nuclear lamina, with lamin B1 facing the inner nuclear membrane, and its outward-facing localization maintains nuclear shape. Conditional Lmnb1 knockout mice showed alterations in myelinating oligodendrocyte populations, indicating lamin B1's importance in regulating oligodendrocyte cell number. Array CGH and real-time PCR analyses revealed that deletions upstream of LMNB1 can lead to ADLD, and an oligodendrocyte-specific silencer element may explain ADLD's central nervous system exclusivity.

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Description

The nuclear lamina, composed of lamins, maintains nuclear shape and function, and perturbations lead to laminopathies, including autosomal dominant...

Technical Information

  • File Format: PDF
  • File Size: 3.62 MB
  • Pages: 118
  • Language: EN
  • Author: Fudrow, John P
  • Total Downloads: 169
  • Last Updated: 1 week ago

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