Transient auditory nerve demyelination as a new mechanism for hidden hearing loss
Hidden hearing loss (HHL) is a recently described auditory neuropathy believed to
contribute to speech discrimination and intelligibility deficits in people with normal …
contribute to speech discrimination and intelligibility deficits in people with normal …
AAV1. NT-3 gene therapy increases muscle fiber diameter through activation of mTOR pathway and metabolic remodeling in a CMT mouse model
ME Yalvac, J Amornvit, L Chen, KM Shontz, S Lewis… - Gene therapy, 2018 - nature.com
Abstract Neurotrophin 3 (NT-3) has well-recognized effects on peripheral nerve and
Schwann cells, promoting axonal regeneration and associated myelination. In this study, we …
Schwann cells, promoting axonal regeneration and associated myelination. In this study, we …
Synergistic PXT3003 therapy uncouples neuromuscular function from dysmyelination in male Charcot–Marie–Tooth disease type 1A (CMT1A) rats
T Prukop, S Wernick, L Boussicault… - Journal of …, 2020 - Wiley Online Library
Abstract Charcot–Marie–Tooth disease 1 A (CMT1A) is caused by an intrachromosomal
duplication of the gene encoding for PMP22 leading to peripheral nerve dysmyelination …
duplication of the gene encoding for PMP22 leading to peripheral nerve dysmyelination …
Rapamycin improves peripheral nerve myelination while it fails to benefit neuromuscular performance in neuropathic mice
Abstract Charcot–-Marie–Tooth disease type 1A (CMT1A) is a hereditary peripheral
neuropathy characterized by progressive demyelination and distal muscle weakness …
neuropathy characterized by progressive demyelination and distal muscle weakness …
Intraepidermal nerve fibre density as biomarker in Charcot–Marie–Tooth disease type 1A
B Hartmannsberger, K Doppler, J Stauber… - Brain …, 2020 - academic.oup.com
Abstract Charcot–Marie–Tooth disease type 1A, caused by a duplication of the gene
peripheral myelin protein 22 kDa, is the most frequent subtype of hereditary peripheral …
peripheral myelin protein 22 kDa, is the most frequent subtype of hereditary peripheral …
Implantable electroceutical approach improves myelination by restoring membrane integrity in a mouse model of peripheral demyelinating neuropathy
Although many efforts are undertaken to treat peripheral demyelinating neuropathies based
on biochemical interventions, unfortunately, there is no approved treatment yet. Furthermore …
on biochemical interventions, unfortunately, there is no approved treatment yet. Furthermore …
Structural and functional abnormalities of the neuromuscular junction in the Trembler-J homozygote mouse model of congenital hypomyelinating neuropathy
AN Scurry, DJ Heredia, CY Feng… - … of Neuropathology & …, 2016 - academic.oup.com
Mutations in peripheral myelin protein 22 (PMP22) result in the most common form of
Charcot-Marie-Tooth (CMT) disease, CMT1A. This hereditary peripheral neuropathy is …
Charcot-Marie-Tooth (CMT) disease, CMT1A. This hereditary peripheral neuropathy is …
Locomotor and skeletal muscle abnormalities in trembler J neuropathic mice
Introduction Patients with hereditary peripheral neuropathies exhibit characteristic
deformities of the hands and feet and have difficulty ambulating. To examine to what extent …
deformities of the hands and feet and have difficulty ambulating. To examine to what extent …
[PDF][PDF] BRAIN COMMUNICATIONS AIN COMMUNICATIONS
B Hartmannsberger, K Doppler, J Stauber… - pdfs.semanticscholar.org
Abbreviations: CIDP ¼ chronic inflammatory demyelinating polyradiculoneuropathy; CMT ¼
Charcot–Marie–Tooth disease; CMT1A ¼ Charcot–Marie–Tooth disease type 1A; CMTNS ¼ …
Charcot–Marie–Tooth disease; CMT1A ¼ Charcot–Marie–Tooth disease type 1A; CMTNS ¼ …
PMP22 Regulates Lipid Metabolism and Cholesterol Trafficking: The Implication for Hereditary Peripheral Neuropathies
Y Zhou - 2019 - search.proquest.com
The misexpression of functional peripheral myelin protein 22 (PMP22) is the major culprit in
hereditary neuropathies, also known as Charcot-Marie-Tooth diseases (CMT). PMP22 is a …
hereditary neuropathies, also known as Charcot-Marie-Tooth diseases (CMT). PMP22 is a …