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
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..View full abstract
This work was supported by the Association Francaise Contre les Myopathies and the National Health and Medical Council (Australia), neither of whom had any role in the study design, collection of data or writing of this report.