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Review
. 2011 Jan 26;31(4):1163-6.
doi: 10.1523/JNEUROSCI.5994-10.2011.

Adult-onset autosomal dominant leukodystrophy: linking nuclear envelope to myelin

Shu-Ting Lin  1 , Louis J Ptácek, Ying-Hui Fu
Affiliations
Review

Adult-onset autosomal dominant leukodystrophy: linking nuclear envelope to myelin

Shu-Ting Lin et al. J Neurosci. .
No abstract available

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Figures

Figure 1.
Figure 1.
T2 weighted MRI showing characteristic white matter hyperintensities identified in patients with Lamin B1 mutations. These MRI abnormalities appear to be restricted to specific white matter tracts and accumulate through the course of disease. A, Transverse (axial) cross section through mid-pons at level of facial colliculus. Short arrow, Corticospinal and corticobulbar tracts (may include crossing pontine tracts); long arrow, medial lemniscus; double arrow, middle cerebellar peduncle (brachium pontis). B, Transverse (axial) cross section through hemispheres at level of lateral ventricles. Three left side arrows, Superior longitudinal fasciculus; short arrow, cingulum. C, Transverse (axial) cross section through caudal midbrain below level of red nucleus. Short arrow, Crossing fibers of superior cerebellar peduncle (brachium conjunctivum); long arrow, medial lemniscus.
Figure 2.
Figure 2.
Schematic representation showing genomic structure of the size and extent of duplications in ADLD. Three families are shown according to size and extent of duplication of LMNB1. Arrows indicate the direction of transcription and red boxes indicate the size and extent of duplications.
Figure 3.
Figure 3.
Level of miR-23 expression in the murine brain gradually increases with age in a pattern that is complementary to that of Lmnb1.
See this image and copyright information in PMC

References

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