Roles of Collagen XXV and Its Putative Receptors PTPσ/δ in Intramuscular Motor Innervation and Congenital Cranial Dysinnervation Disorder.
Animals
Axons
/ metabolism
Cell Line
Diaphragm
/ abnormalities
Disease Models, Animal
Gene Expression Regulation
HEK293 Cells
Humans
Mice
Mice, Knockout
Motor Neurons
/ metabolism
Muscle, Skeletal
/ abnormalities
Myoblasts
/ metabolism
Non-Fibrillar Collagens
/ genetics
Ocular Motility Disorders
/ congenital
Protein Binding
Protein Tyrosine Phosphatases, Non-Receptor
/ genetics
Receptor-Like Protein Tyrosine Phosphatases, Class 2
/ genetics
Sciatic Nerve
/ injuries
Sciatic Neuropathy
/ surgery
Signal Transduction
Skull
/ abnormalities
collagen
motor neuron
neuromuscular development
receptor protein tyrosine phosphatase
Journal
Cell reports
ISSN: 2211-1247
Titre abrégé: Cell Rep
Pays: United States
ID NLM: 101573691
Informations de publication
Date de publication:
24 Dec 2019
24 Dec 2019
Historique:
received:
15
03
2019
revised:
23
09
2019
accepted:
26
11
2019
entrez:
26
12
2019
pubmed:
26
12
2019
medline:
25
9
2020
Statut:
ppublish
Résumé
Intramuscular motor innervation is an essential process in neuromuscular development. Recently, mutations in COL25A1, encoding CLAC-P/collagen XXV, have been linked to the development of a congenital cranial dysinnervation disorder (CCDD). Yet the molecular mechanisms of intramuscular innervation and the etiology of CCDD related to COL25A1 have remained elusive. Here, we report that muscle-derived collagen XXV is indispensable for intramuscular innervation. In developing skeletal muscles, Col25a1 expression is tightly regulated by muscle excitation. In vitro and cell-based assays reveal a direct interaction between collagen XXV and receptor protein tyrosine phosphatases (PTPs) σ and δ. Motor explant assays show that expression of collagen XXV in target cells attracts motor axons, but this is inhibited by exogenous PTPσ/δ. CCDD mutations attenuate motor axon attraction by reducing collagen XXV-PTPσ/δ interaction. Overall, our study identifies PTPσ/δ as putative receptors for collagen XXV, implicating collagen XXV and PTPσ/δ in intramuscular innervation and a developmental ocular motor disorder.
Identifiants
pubmed: 31875546
pii: S2211-1247(19)31635-3
doi: 10.1016/j.celrep.2019.11.112
pii:
doi:
Substances chimiques
Non-Fibrillar Collagens
0
Protein Tyrosine Phosphatases, Non-Receptor
EC 3.1.3.48
Ptpn21 protein, mouse
EC 3.1.3.48
Ptprs protein, mouse
EC 3.1.3.48
Receptor-Like Protein Tyrosine Phosphatases, Class 2
EC 3.1.3.48
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
4362-4376.e6Informations de copyright
Copyright © 2019 The Author(s). Published by Elsevier Inc. All rights reserved.