Construction of a mouse model of Charcot-Marie-Tooth disease type 1A by pronuclear injection of human YAC DNA.
about
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 mouseLate-onset neurodegeneration in mice with increased dosage of the proteolipid protein genePMP22 transgenic dorsal root ganglia cultures show myelin abnormalities similar to those of human CMT1ACollagen XXVIII is a distinctive component of the peripheral nervous system nodes of ranvier and surrounds nonmyelinating glial cellsFunctional and comparative genomics analyses of pmp22 in medaka fishCharcot-Marie-Tooth disease: lessons in genetic mechanisms.Charcot-Marie-Tooth polyneuropathy: duplication, gene dosage, and genetic heterogeneity.Charcot-marie-tooth disease and related neuropathies: molecular basis for distinction and diagnosis.CamKII inhibitors reduce mitotic instability, connexon anomalies and progression of the in vivo behavioral phenotype in transgenic animals expressing a mutated Gjb1 gene.Introduction to the Third International Symposium on Charcot-Marie-Tooth disorders.Molecular mechanisms for CMT1A duplication and HNPP deletion.Distinct phenotypes associated with increasing dosage of the PLP gene: implications for CMT1A due to PMP22 gene duplication.Transgenic mouse models of CMT1A and HNPP.The "CMT rat": peripheral neuropathy and dysmyelination caused by transgenic overexpression of PMP22.Human nerve pathology caused by different mutational mechanisms of the PMP22 gene.The PMP22 gene and its related diseases.Copy number variation upstream of PMP22 in Charcot-Marie-Tooth diseaseAn essential role of MAG in mediating axon-myelin attachment in Charcot-Marie-Tooth 1A diseaseNerve-dependent changes in skeletal muscle myosin heavy chain after experimental denervation and cross-reinnervation and in a demyelinating mouse model of Charcot-Marie-Tooth disease type 1A.Genome editing-enabled HTS assays expand drug target pathways for Charcot-Marie-tooth disease.Understanding Schwann cell-neurone interactions: the key to Charcot-Marie-Tooth disease?Regulation of the PMP22 gene through an intronic enhancer.Mouse molecular cytogenetic resource: 157 BACs link the chromosomal and genetic maps.Pharmacological induction of the heat shock response improves myelination in a neuropathic modelGenetic aspects of Charcot-Marie-Tooth disease.Distal enhancers upstream of the Charcot-Marie-Tooth type 1A disease gene PMP22Rapamycin activates autophagy and improves myelination in explant cultures from neuropathic mice.Role of immune cells in animal models for inherited peripheral neuropathies.Animal models of Charcot-Marie-Tooth disease type 1A.Pathomechanisms of mutant proteins in Charcot-Marie-Tooth disease.Molecular mechanisms of inherited demyelinating neuropathies.Genetic deletion of Cadm4 results in myelin abnormalities resembling Charcot-Marie-Tooth neuropathy.Biochemical characterization of protein quality control mechanisms during disease progression in the C22 mouse model of CMT1A.Intermittent fasting alleviates the neuropathic phenotype in a mouse model of Charcot-Marie-Tooth disease.Charcot-marie-tooth disease: seventeen causative genes.Murine therapeutic models for Charcot-Marie-Tooth (CMT) disease.Therapeutic options in Charcot-Marie-Tooth diseases.Exposure at the cell surface is required for gas3/PMP22 To regulate both cell death and cell spreading: implication for the Charcot-Marie-Tooth type 1A and Dejerine-Sottas diseases.Rho-dependent regulation of cell spreading by the tetraspan membrane protein Gas3/PMP22.MCP-1/CCL2 modifies axon properties in a PMP22-overexpressing mouse model for Charcot-Marie-tooth 1A neuropathy.
P2860
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P2860
Construction of a mouse model of Charcot-Marie-Tooth disease type 1A by pronuclear injection of human YAC DNA.
description
1996 nî lūn-bûn
@nan
1996年の論文
@ja
1996年学术文章
@wuu
1996年学术文章
@zh-cn
1996年学术文章
@zh-hans
1996年学术文章
@zh-my
1996年学术文章
@zh-sg
1996年學術文章
@yue
1996年學術文章
@zh
1996年學術文章
@zh-hant
name
Construction of a mouse model ...... ar injection of human YAC DNA.
@en
Construction of a mouse model ...... ar injection of human YAC DNA.
@nl
type
label
Construction of a mouse model ...... ar injection of human YAC DNA.
@en
Construction of a mouse model ...... ar injection of human YAC DNA.
@nl
prefLabel
Construction of a mouse model ...... ar injection of human YAC DNA.
@en
Construction of a mouse model ...... ar injection of human YAC DNA.
@nl
P2093
P356
P1476
Construction of a mouse model ...... ar injection of human YAC DNA.
@en
P2093
Figarella-Branger D
Pellissier JF
P304
P356
10.1093/HMG/5.5.563
P577
1996-05-01T00:00:00Z