×
Entrez Id: 9927
Gene Symbol: MFN2
MFN2
Dejerine-Sottas Disease (disorder)
0.200
Biomarker
MGD
×
Entrez Id: 4359
Gene Symbol: MPZ
MPZ
Dejerine-Sottas Disease (disorder)
1.000
Biomarker
MGD
Mouse P0 gene disruption leads to hypomyelination, abnormal expression of recognition molecules, and degeneration of myelin and axons.
1384988 1992
×
Entrez Id: 4359
Gene Symbol: MPZ
MPZ
Dejerine-Sottas Disease (disorder)
1.000
Biomarker
MGD
Protein zero (P0)-deficient mice show myelin degeneration in peripheral nerves characteristic of inherited human neuropathies.
7581451 1995
×
Entrez Id: 2705
Gene Symbol: GJB1
GJB1
Dejerine-Sottas Disease (disorder)
0.630
Biomarker
MGD
Defective propagation of signals generated by sympathetic nerve stimulation in the liver of connexin32-deficient mice.
8790370 1996
×
Entrez Id: 5376
Gene Symbol: PMP22
PMP22
Dejerine-Sottas Disease (disorder)
1.000
Biomarker
MGD
Abnormal Schwann cell/axon interactions in the Trembler-J mouse.
9147228 1997
×
Entrez Id: 2705
Gene Symbol: GJB1
GJB1
Dejerine-Sottas Disease (disorder)
0.630
Biomarker
MGD
Structural abnormalities and deficient maintenance of peripheral nerve myelin in mice lacking the gap junction protein connexin 32.
9169515 1997
×
Entrez Id: 2705
Gene Symbol: GJB1
GJB1
Dejerine-Sottas Disease (disorder)
0.630
Biomarker
MGD
Connexin32-null mice develop demyelinating peripheral neuropathy.
9700485 1998
×
Entrez Id: 4000
Gene Symbol: LMNA
LMNA
Dejerine-Sottas Disease (disorder)
0.200
Biomarker
MGD
Loss of A-type lamin expression compromises nuclear envelope integrity leading to muscular dystrophy.
10579712 1999
×
Entrez Id: 23095
Gene Symbol: KIF1B
KIF1B
Dejerine-Sottas Disease (disorder)
0.230
Biomarker
MGD
Charcot-Marie-Tooth disease type 2A caused by mutation in a microtubule motor KIF1Bbeta.
11389829 2001
×
Entrez Id: 9990
Gene Symbol: SLC12A6
SLC12A6
Dejerine-Sottas Disease (disorder)
0.200
Biomarker
MGD
The K-Cl cotransporter KCC3 is mutant in a severe peripheral neuropathy associated with agenesis of the corpus callosum.
12368912 2002
×
Entrez Id: 4000
Gene Symbol: LMNA
LMNA
Dejerine-Sottas Disease (disorder)
0.200
Biomarker
MGD
Homozygous defects in LMNA, encoding lamin A/C nuclear-envelope proteins, cause autosomal recessive axonal neuropathy in human (Charcot-Marie-Tooth disorder type 2) and mouse.
11799477 2002
×
Entrez Id: 9990
Gene Symbol: SLC12A6
SLC12A6
Dejerine-Sottas Disease (disorder)
0.200
Biomarker
MGD
Loss of K-Cl co-transporter KCC3 causes deafness, neurodegeneration and reduced seizure threshold.
14532115 2003
×
Entrez Id: 5376
Gene Symbol: PMP22
PMP22
Dejerine-Sottas Disease (disorder)
1.000
Biomarker
MGD
Nerve conduction abnormalities in the trembler-j mouse: a model for Charcot-Marie-Tooth disease type 1A?
15363066 2004
×
Entrez Id: 10397
Gene Symbol: NDRG1
NDRG1
Dejerine-Sottas Disease (disorder)
0.200
Biomarker
MGD
Ndrg1-deficient mice exhibit a progressive demyelinating disorder of peripheral nerves.
15082788 2004
×
Entrez Id: 8898
Gene Symbol: MTMR2
MTMR2
Dejerine-Sottas Disease (disorder)
0.200
Biomarker
MGD
Disruption of Mtmr2 produces CMT4B1-like neuropathy with myelin outfolding and impaired spermatogenesis.
15557122 2004
×
Entrez Id: 5376
Gene Symbol: PMP22
PMP22
Dejerine-Sottas Disease (disorder)
1.000
Biomarker
MGD
Altered ion channels in an animal model of Charcot-Marie-Tooth disease type IA.
15703401 2005
×
Entrez Id: 5376
Gene Symbol: PMP22
PMP22
Dejerine-Sottas Disease (disorder)
1.000
Biomarker
MGD
Impaired proteasome activity and accumulation of ubiquitinated substrates in a hereditary neuropathy model.
15748170 2005
×
Entrez Id: 1959
Gene Symbol: EGR2
EGR2
Dejerine-Sottas Disease (disorder)
0.940
Biomarker
MGD
Analysis of congenital hypomyelinating Egr2Lo/Lo nerves identifies Sox2 as an inhibitor of Schwann cell differentiation and myelination.
15695336 2005
×
Entrez Id: 1959
Gene Symbol: EGR2
EGR2
Dejerine-Sottas Disease (disorder)
0.940
Biomarker
MGD
Nab proteins are essential for peripheral nervous system myelination.
16136673 2005
×
Entrez Id: 8898
Gene Symbol: MTMR2
MTMR2
Dejerine-Sottas Disease (disorder)
0.200
Biomarker
MGD
An animal model for Charcot-Marie-Tooth disease type 4B1.
16249189 2005
×
Entrez Id: 2617
Gene Symbol: GARS1
GARS1
Dejerine-Sottas Disease (disorder)
0.200
Biomarker
MGD
An active dominant mutation of glycyl-tRNA synthetase causes neuropathy in a Charcot-Marie-Tooth 2D mouse model.
16982418 2006
×
Entrez Id: 5376
Gene Symbol: PMP22
PMP22
Dejerine-Sottas Disease (disorder)
1.000
Biomarker
MGD
Oral curcumin mitigates the clinical and neuropathologic phenotype of the Trembler-J mouse: a potential therapy for inherited neuropathy.
17701891 2007
×
Entrez Id: 5376
Gene Symbol: PMP22
PMP22
Dejerine-Sottas Disease (disorder)
1.000
Biomarker
MGD
The formation of peripheral myelin protein 22 aggregates is hindered by the enhancement of autophagy and expression of cytoplasmic chaperones.
17174099 2007
×
Entrez Id: 81846
Gene Symbol: SBF2
SBF2
Dejerine-Sottas Disease (disorder)
0.200
Biomarker
MGD
Mtmr13/Sbf2-deficient mice: an animal model for CMT4B2.
17855448 2007
×
Entrez Id: 9896
Gene Symbol: FIG4
FIG4
Dejerine-Sottas Disease (disorder)
0.200
Biomarker
MGD
Mutation of FIG4 causes neurodegeneration in the pale tremor mouse and patients with CMT4J.
17572665 2007