Year: 2021
Type: article
Abstract: The neuromuscular synapse, neuromuscular junction （NMJ）, is essential for survival. Acetylcholine receptor clustering on the postsynaptic membrane is a cardinal event in the differentiation of NMJ. The neuron-secreted agrin and its coreceptors low-density lipoprotein-receptor related protein （LRP4） and muscle-specific tyrosine kinase （MuSK） are critical for NMJ formation. LRP4 is a type Ⅰ single transmembrane protein. In muscle, LRP4 serves as a receptor for agrin and is essential for the activation of MuSK and the clustering of acetylcholine receptors. In this review, I discuss the structural basis for NMJ, the abnormality of Lrp4 -decifient mice and the LRP4 -related diseases. I also present our publications: 1） Amniotic fluid volume during mid-to-late gestation is known to depend mainly on the urine excretion from the fetal kidneys and partly on the fluid secretion from the fetal lungs during fetal breathing-like movements. The loss of Lrp4 compromised fetal swallowing and breathing-like movements, resulted in polyhydramnios despite the impairment of urine production. This findings indicate that amniotic fluid removal plays an essential role in regulating the amniotic fluid volume at perinatal stage. 2） The loss of Lrp4 resulted in an elevated incidence of microphthalmia and enhanced the incidence of aberrant retinal folds.
Author: TANAHASHI, Hiroshi
Institution: Tokiwa University
Language: Japanese
Subfield: Molecular Biology
Domain: Life Sciences
Open Access status: green
OpenAlex ID: W7146660252