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Article

Degree of Cajal-Retzius Cell Mislocalization Correlates with the Severity of Structural Brain Defects in Mouse Models of Dystroglycanopathy.

Brain pathology (Zurich, Switzerland) - 1 Jul 2016

Booler Helen S, Williams Josie L, Hopkinson Mark, Brown Susan C

Abstract excerpt

The secondary dystroglycanopathies are characterized by the hypoglycosylation of alpha dystroglycan, and are associated with mutations in at least 18 genes that act on the glycosylation of this cell surface receptor rather than the Dag1 gene itself. At the severe end of the disease spectrum, there are substantial structural brain defects, the most striking of which is often cobblestone lissencephaly. The aim of...

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Identifiers

PMID: 26306834
DOI: 10.1111/bpa.12306
PMCID: PMC8029081

Genes

FKRP

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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