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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