RESCUE: Interdependent Challenges of Reliability, Security and Quality in Nanoelectronic Systems
(2019)
Guanosine diphosphate-mannose pyrophosphorylase B (GMPPB) catalyzes the conversion of mannose-1-phosphate and GTP to GDP-mannose, which is required as a mannose donor for the biosynthesis of glycan structures necessary for proper cellular functions. Mutations in GMPPB have been associated with variable neuromuscular disorders such as muscular dystrophy and myasthenic
syndromes. Here, we report that GMPPB protein abundance increases during brain and skeletal muscle development, which is accompanied by an increase in overall protein mannosylation. To model the human disorder in mice, we generated heterozygous GMPPB KO mice using CRISPR/Cas9. While we were able to obtain homozygous KO mice from heterozygous matings at the blastocyst stage, homozygous KO embryos were absent beyond embryonic day E8.5, suggesting that the homozygous loss of GMPPB results in early embryonic lethality. Since patients with GMPPB loss-of-function manifest with neuromuscular disorders, we investigated the role of GMPPB in vitro. Thereby, we found that the siRNA-mediated knockdown of Gmppb in either primary
myoblasts or the myoblast cell line C2C12 impaired myoblast differentiation and resulted in myotube degeneration. siRNA-mediated knockdown of Gmppb also impaired the neuron-like differentiation of N2A cells. Taken together, our data highlight the essential role of GMPPB during development and differentiation, especially in myogenic and neuronal cell types.
Linear woody-features, such as hedgerows, windbreaks, and riparian buffer strips, composed of trees and/or shrubs are anthropogenic features, established in the past for different purposes, such as provision of field boundaries, protection from wind and supply of fuelwood. Today, they are primarily valued for their ecological benefit, while their production function has received rather little attention. This study assessed the biomass potential of existing linear woodyfeatures in a study area in southern Brandenburg, Germany. The merchantable tree volume of the measured woody-features ranged between 240 m3 ha-1 and 710 m3 ha-1, depending on the relative proportion of trees and shrubs. The results suggest that the biomass potential of linear woody-features with predominant tree proportion per hectare can be higher than this of forests.
A strategy for utilising the production function of these woody-features should take into account the provision of benefits such as wind protection, habitat provision and landscape aesthetics.