Shiverer mouse

Shiverer mice are mice which possess the shiverer (shi/shi) mutation in the MBP gene. Shiverer mice develop a characteristic "shaking" or "shivering" gait within a few weeks of birth. They are commonly used as animal models of leukodystrophy in neuroscience research.

Characteristics

The shiverer mutation is an autosomal recessive loss-of-function mutation. It was generated by a 20-kilobase deletion within the MBP gene,[1] resulting in the failure of oligodendrocytes to form compact myelin in the central nervous system. Axons in shiverer mice fail to attain a normal diameter and exhibit altered cytoskeleton structure.[2]

Approximately twelve days after birth, shiverer mice begin to exhibit tremors which progressively worsen. They exhibit tonic seizure behavior after weaning and die prematurely, typically between 50 and 100 days after birth.[3]

Research use

Shiverer mice are used to model human leukodystrophies due to the congenital nature and early onset of the shiverer phenotype, which mimics the disease progression observed in humans. They are also used to study myelin defects more generally in demyelinating diseases such as multiple sclerosis.

The shiverer model has also been used to test novel therapies which promote myelination such as neural stem cell therapy.[4]

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References
  1. Molineaux SM, Engh H, de Ferra F, Hudson L, Lazzarini RA (1986). "Recombination within the myelin basic protein gene created the dysmyelinating shiverer mouse mutation". Proc Natl Acad Sci U S A. 83 (19): 7542–7546. Bibcode:1986PNAS...83.7542M. doi:10.1073/pnas.83.19.7542. PMC 386755. PMID 2429310.CS1 maint: multiple names: authors list (link)
  2. Johansen-Berg H, Behrens TEJ (2014). "Chapter 7. White Matter Structure: A Microscopist's View". Diffusion MRI (2 ed.). pp. 127–153. doi:10.1016/B978-0-12-396460-1.00007-X.
  3. Readhead C, Hood L (1990). "The dysmyelinating mouse mutations shiverer (shi) and myelin deficient (shi mld)". Behav Genet. 20 (2): 213–234. doi:10.1007/BF01067791. PMID 1693848.
  4. Baker M (12 June 2008). "Human cell transplants stop the shivers". Nature Reports Stem Cells. doi:10.1038/stemcells.2008.92.
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