×
Entrez Id:
4359
Gene Symbol:
MPZ
MPZ
Hereditary Motor and Sensory Neuropathies
0.600
Biomarker
MGD
A nonsense mutation in myelin protein zero causes congenital hypomyelination neuropathy through altered P0 membrane targeting and gain of abnormal function.
30239779
2019
×
Entrez Id:
4359
Gene Symbol:
MPZ
MPZ
Hereditary Motor and Sensory Neuropathies
0.600
Biomarker
MGD
MpzR98C arrests Schwann cell development in a mouse model of early-onset Charcot-Marie-Tooth disease type 1B.
22689911
2012
×
Entrez Id:
4359
Gene Symbol:
MPZ
MPZ
Hereditary Motor and Sensory Neuropathies
0.600
Biomarker
MGD
Monocyte chemoattractant protein-1 is a pathogenic component in a model for a hereditary peripheral neuropathy.
18326085
2008
×
Entrez Id:
5376
Gene Symbol:
PMP22
PMP22
Hereditary Motor and Sensory Neuropathies
0.600
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
Hereditary Motor and Sensory Neuropathies
0.600
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:
5376
Gene Symbol:
PMP22
PMP22
Hereditary Motor and Sensory Neuropathies
0.600
Biomarker
MGD
Impaired proteasome activity and accumulation of ubiquitinated substrates in a hereditary neuropathy model.
15748170
2005
×
Entrez Id:
5376
Gene Symbol:
PMP22
PMP22
Hereditary Motor and Sensory Neuropathies
0.600
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
Hereditary Motor and Sensory Neuropathies
0.600
Biomarker
MGD
Nerve conduction abnormalities in the trembler-j mouse: a model for Charcot-Marie-Tooth disease type 1A?
15363066
2004
×
Entrez Id:
5376
Gene Symbol:
PMP22
PMP22
Hereditary Motor and Sensory Neuropathies
0.600
Biomarker
MGD
Abnormal Schwann cell/axon interactions in the Trembler-J mouse.
9147228
1997
×
Entrez Id:
4359
Gene Symbol:
MPZ
MPZ
Hereditary Motor and Sensory Neuropathies
0.600
Biomarker
MGD
Protein zero (P0)-deficient mice show myelin degeneration in peripheral nerves characteristic of inherited human neuropathies.
7581451
1995
×
Entrez Id:
4359
Gene Symbol:
MPZ
MPZ
Hereditary Motor and Sensory Neuropathies
0.600
Biomarker
MGD
Mouse P0 gene disruption leads to hypomyelination, abnormal expression of recognition molecules, and degeneration of myelin and axons.
1384988
1992
×
Entrez Id:
1959
Gene Symbol:
EGR2
EGR2
Hereditary Motor and Sensory Neuropathies
0.500
Biomarker
MGD
Congenital hypomyelinating neuropathy with lethal conduction failure in mice carrying the Egr2 I268N mutation.
19244508
2009
×
Entrez Id:
1959
Gene Symbol:
EGR2
EGR2
Hereditary Motor and Sensory Neuropathies
0.500
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
Hereditary Motor and Sensory Neuropathies
0.500
Biomarker
MGD
Nab proteins are essential for peripheral nervous system myelination.
16136673
2005
×
Entrez Id:
1778
Gene Symbol:
DYNC1H1
DYNC1H1
Hereditary Motor and Sensory Neuropathies
0.310
Biomarker
MGD
A novel mouse model carrying a human cytoplasmic dynein mutation shows motor behavior deficits consistent with Charcot-Marie-Tooth type 2O disease.
29379136
2018
×
Entrez Id:
2705
Gene Symbol:
GJB1
GJB1
Hereditary Motor and Sensory Neuropathies
0.270
Biomarker
MGD
Connexin32-null mice develop demyelinating peripheral neuropathy.
9700485
1998
×
Entrez Id:
2705
Gene Symbol:
GJB1
GJB1
Hereditary Motor and Sensory Neuropathies
0.270
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
Hereditary Motor and Sensory Neuropathies
0.270
Biomarker
MGD
Defective propagation of signals generated by sympathetic nerve stimulation in the liver of connexin32-deficient mice.
8790370
1996
×
Entrez Id:
10397
Gene Symbol:
NDRG1
NDRG1
Hereditary Motor and Sensory Neuropathies
0.250
Biomarker
MGD
Ndrg1 in development and maintenance of the myelin sheath.
21303696
2011
×
Entrez Id:
10397
Gene Symbol:
NDRG1
NDRG1
Hereditary Motor and Sensory Neuropathies
0.250
Biomarker
MGD
Ndrg1-deficient mice exhibit a progressive demyelinating disorder of peripheral nerves.
15082788
2004
×
Entrez Id:
9927
Gene Symbol:
MFN2
MFN2
Hereditary Motor and Sensory Neuropathies
0.240
Biomarker
MGD
×
Entrez Id:
8898
Gene Symbol:
MTMR2
MTMR2
Hereditary Motor and Sensory Neuropathies
0.230
Biomarker
MGD
An animal model for Charcot-Marie-Tooth disease type 4B1.
16249189
2005
×
Entrez Id:
8898
Gene Symbol:
MTMR2
MTMR2
Hereditary Motor and Sensory Neuropathies
0.230
Biomarker
MGD
Disruption of Mtmr2 produces CMT4B1-like neuropathy with myelin outfolding and impaired spermatogenesis.
15557122
2004
×
Entrez Id:
23095
Gene Symbol:
KIF1B
KIF1B
Hereditary Motor and Sensory Neuropathies
0.230
Biomarker
MGD
Charcot-Marie-Tooth disease type 2A caused by mutation in a microtubule motor KIF1Bbeta.
11389829
2001
×
Entrez Id:
54332
Gene Symbol:
GDAP1
GDAP1
Hereditary Motor and Sensory Neuropathies
0.220
Biomarker
MGD
Lack of GDAP1 induces neuronal calcium and mitochondrial defects in a knockout mouse model of charcot-marie-tooth neuropathy.
25860513
2015