Precision mouse models of Yars/dominant intermediate Charcot-Marie-Tooth disease type C and Sptlc1/hereditary sensory and autonomic neuropathy type 1.

Document Type

Article

Publication Date

11-1-2022

Publication Title

Journal of anatomy

Keywords

JMG, Animals, Charcot-Marie-Tooth Disease, Disease Models, Animal, Hereditary Sensory and Autonomic Neuropathies, Humans, Ligases, Mice, Mutation, Peripheral Nervous System Diseases, RNA, Transfer, Serine C-Palmitoyltransferase

JAX Source

J Anat. 2022;241(5):1169-85.

Volume

241

Issue

5

First Page

1169

Last Page

1185

ISSN

1469-7580

PMID

34875719

DOI

https://doi.org/10.1111/joa.13605

Grant

National Institutes of Health, Grant/ Award Number: R37 NS054154, R24 NS098523, R01 NS113583 and CA34196; Swiss National Science Foundation, Grant/ Award Number: SNF 31003A_179371; European Joint Programme on Rare Diseases, Grant/Award Number: EJP RD+SNF 32ER30_187505

Abstract

Animal models of neurodegenerative diseases such as inherited peripheral neuropathies sometimes accurately recreate the pathophysiology of the human disease, and sometimes accurately recreate the genetic perturbations found in patients. Ideally, models achieve both, but this is not always possible; nonetheless, such models are informative. Here we describe two animal models of inherited peripheral neuropathy: mice with a mutation in tyrosyl tRNA-synthetase, Yars

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