Ndrg1-Deficient Mice Exhibit a Progressive Demyelinating Disorder of Peripheral Nerves
Copyright policy )Ndrg1-Deficient Mice Exhibit a Progressive Demyelinating Disorder of Peripheral Nerves
NDRG1 is an intracellular protein that is induced under a number of stress and pathological conditions, and it is thought to be associated with cell growth and differentiation. Recently, human NDRG1 was identified as a gene responsible for hereditary motor and sensory neuropathy-Lom (classified as Charcot-Marie-Tooth disease type 4D), which is characterized by early-onset peripheral neuropathy, leading to severe disability in adulthood. In this study, we generated mice lacking Ndrg1 to analyze its function and elucidate the pathogenesis of Charcot-Marie-Tooth disease type 4D. Histological analysis showed that the sciatic nerve of Ndrg1-deficient mice degenerated with demyelination at about 5 weeks of age. However, myelination of Schwann cells in the sciatic nerve was normal for 2 weeks after birth. Ndrg1-deficient mice showed muscle weakness, especially in the hind limbs, but complicated motor skills were retained. In wild-type mice, NDRG1 was abundantly expressed in the cytoplasm of Schwann cells rather than the myelin sheath. These results indicate that NDRG1 deficiency leads to Schwann cell dysfunction, suggesting that NDRG1 is essential for maintenance of the myelin sheaths in peripheral nerves. These mice will be used for future analyses of the mechanisms of myelin maintenance.
- Osaka University Japan
Adult, Male, Mice, Knockout, Body Weight, Intracellular Signaling Peptides and Proteins, Cell Cycle Proteins, Motor Activity, Sciatic Nerve, Mice, Phenotype, Charcot-Marie-Tooth Disease, Animals, Humans, Tissue Distribution, Peripheral Nerves, Schwann Cells, Muscle, Skeletal, Myelin Sheath, Demyelinating Diseases, Muscle Contraction
Adult, Male, Mice, Knockout, Body Weight, Intracellular Signaling Peptides and Proteins, Cell Cycle Proteins, Motor Activity, Sciatic Nerve, Mice, Phenotype, Charcot-Marie-Tooth Disease, Animals, Humans, Tissue Distribution, Peripheral Nerves, Schwann Cells, Muscle, Skeletal, Myelin Sheath, Demyelinating Diseases, Muscle Contraction
citations This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).130 popularity This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.Top 10% influence This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).Top 10% impulse This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.Top 10%
Citations provided by BIP!Popularity provided by BIP!