Shiverer mouse

Randykitty: added Category:Behavioural genetics using HotCat

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'''''Shiverer'' mice''' are [[house mouse|mice]] which possess the ''shiverer'' (shi/shi) mutation in the [[myelin basic protein|''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 [[dominance (genetics)|autosomal recessive]] [[Mutation#By_effect_on_function|loss-of-function]] mutation. It was generated by a 20-kilobase deletion within the [[myelin basic protein|''MBP'']] gene,<ref name="pmid2429310"></ref> resulting in the failure of [[oligodendrocyte]]s to form compact [[myelin]] in the [[central nervous system]]. [[Axon]]s in ''shiverer'' mice fail to attain a normal diameter and exhibit altered [[cytoskeleton]] structure.<ref name="DiffusionMRI"></ref>

Approximately twelve days after birth, ''shiverer'' mice begin to exhibit [[tremor]]s which progressively worsen. They exhibit [[tonic seizure]] behavior after weaning and die prematurely, typically between 50 and 100 days after birth.<ref name="pmid1693848"></ref>

==Research use==
''Shiverer'' mice are used to model human [[leukodystrophy|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 disease]]s such as [[multiple sclerosis]].

The ''shiverer'' model has also been used to test novel therapies which promote [[myelination]] such as [[neural stem cell]] therapy.<ref name="baker2008">Liquid error: wrong number of arguments (1 for 2)</ref>

==References==


[[Category:Laboratory mouse strains]]
[[Category:Animal models in neuroscience]]
[[Category:Behavioural genetics]]

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