Stock B6;CBACa-Tg(PMP22)C22Clh/H
FESA Number
784
Strain Name
B6;CBACa-Tg(PMP22)C22Clh/H
Mutation Strain Type
| Mutation Type | Mutation Subtype | Strain Type |
|---|---|---|
| IMSR - Transgenic | IMSR - unclassified |
Attribution
| Institution name | Depositor / Originator | Name |
|---|---|---|
| Imperial College | Originator | Retained |
| Imperial College | Depositor | Retained |
Gene/Allele Information
| Allele Name | Allele MGI ID | Gene Name | Gene MGI ID | Chromosome |
|---|---|---|---|---|
| Tg(PMP22)C22Clh | MGI:2183770 | 8 |
Severity Limit
Substantial
Phenotypic Description
Shaking & unsteady gait. Males rarely breed, females may have several litters before becoming too disabled.
Publications
Displaying 1 - 10 of 10 Stock Publications
| Pubmed ID | Authors | Title | Journal |
|---|---|---|---|
| 19111616 | Barbaria EM | The alpha-chemokine CXCL14 is up-regulated in the sciatic nerve of a mouse model of Charcot-Marie-Tooth disease type 1A and alters myelin gene expression in cultured Schwann cells. | Neurobiol Dis (2 009) 33:448-58 |
| 16326107 | Fortun J | Alterations in degradative pathways and protein aggregation in a neuropathy model based on PMP22 overexpression. | Neurobiol Dis (2 006) 22:153-64 |
| 20830809 | Grimal S | Collagen XXVIII is a distinctive component of the peripheral nervous system nodes of ranvier and surrounds nonmyelinating glial cells. | Glia (2 010) 58:1977-87 |
| 9467003 | Huxley C | Correlation between varying levels of PMP22 expression and the degree of demyelination and reduction in nerve conduction velocity in transgenic mice. | Hum Mol Genet (1 998) 7:449-58 |
| 8733121 | Huxley C | Construction of a mouse model of Charcot-Marie-Tooth disease type 1A by pronuclear injection of human YAC DNA. | Hum Mol Genet (1 996) 5:563-9 |
| 24526449 | Klein D | Nonuniform molecular features of myelinating Schwann cells in models for CMT1: distinct disease patterns are associated with NCAM and c-Jun upregulation. | Glia (2 014) 62:736-50 |
| 11328356 | Norreel JC | Behavioural profiling of a murine Charcot-Marie-Tooth disease type 1A model. | Eur J Neurosci (2 001) 13:1625-34 |
| 12573712 | Norreel JC | Close relationship between motor impairments and loss of functional motoneurons in a Charcot-Marie-Tooth type 1A model. | Neuroscience (2 003) 116:695-703 |
| 18655835 | Rangaraju S | Pharmacological induction of the heat shock response improves myelination in a neuropathic model. | Neurobiol Dis (2 008) 32:105-15 |
| 10586249 | Suter U | Transgenic mouse models of CMT1A and HNPP. | Ann N Y Acad Sci (1 999) 883:247-53 |
EMMA ID
EM:01883
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