Journal article

Ndrg1 in development and maintenance of the myelin sheath

Rosalind HM King, David Chandler, Sash Lopaticki, Dexing Huang, Julian Blake, John R Muddle, Trevor Kilpatrick, Michelle Nourallah, Toshiyuki Miyata, Tomohiko Okuda, Kim W Carter, Michael Hunter, Dora Angelicheva, Grant Morahan, Luba Kalaydjieva

Neurobiology of Disease | ACADEMIC PRESS INC ELSEVIER SCIENCE | Published : 2011

Abstract

CMT4D disease is a severe autosomal recessive demyelinating neuropathy with extensive axonal loss leading to early disability, caused by mutations in the N-myc downstream regulated gene 1 (NDRG1). NDRG1 is expressed at particularly high levels in the Schwann cell (SC), but its physiological function(s) are unknown. To help with their understanding, we characterise the phenotype of a new mouse model, stretcher (str), with total Ndrg1 deficiency, in comparison with the hypomorphic Ndrg1 knock-out (KO) mouse. While both models display normal initial myelination and a transition to overt pathology between weeks 3 and 5, the markedly more severe str phenotype suggests that even low Ndrg1 expressi..

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