Fundamental Pharma GmbH has raised €10 million (US$10.3 million) in a seed round to develop a new class of glutamate inhibitors, after uncovering a route to maintaining the protective effects of the neurotransmitter in the synapses while preventing neurotoxicity when it is released elsewhere.

The mutant gene causing Huntington’s disease (HD) is active from the earliest stages of brain development, even though the pathology is not evident until between 30 and 50 years of age. That delay is ascribed to plasticity enabling the brain to compensate to such an extent that overt signs of disease take time to develop. As a result, it is difficult to plot a route from early molecular defects to development of HD several decades later.

Age-related diseases have been explained as due in part to the excessive generation and accumulation of waste products like the various insoluble protein aggregates observed in nondividing neurons of Alzheimer’s disease, Parkinson’s disease, amyotrophic lateral sclerosis and Huntington’s disease.

Connections, Susan Greenfield told her audience at the 2022 Annual Conference of the European Academy of Neurology, are what the mind is all about. "When you are born, you are born with a fair amount of neurons," she said at the conference's opening plenary on Sunday. But "what characterizes the growth of the brain postnatally is the configurations of connections."

Researchers report in the June 21, 2021, online issue of Neuron that overexpression of the LDL receptor can reduce ApoE to prevent tauopathy-associated neurodegeneration in mouse models.

Researchers at the University of Queensland Brain Institute have for the first time shown that a proteotoxic species can increase mitochondrial DNA mutations in neurons.

Collectively, lysosomal storage disorders (LSDs) are caused by malfunctions in metabolic enzymes in the lysosome system. Depending on which enzyme is missing, toxic metabolites accumulate. While the LSDs are highly heterogenous – even within one disease, presentation can vary widely – neurodegeneration is a common feature in these disorders.

Collectively, lysosomal storage disorders (LSDs) are caused by malfunctions in metabolic enzymes in the lysosome system. Depending on which enzyme is missing, toxic metabolites accumulate. While the LSDs are highly heterogenous – even within one disease, presentation can vary widely – neurodegeneration is a common feature in these disorders.

Using the roundworm C. elegans as a "living test tube," researchers at the University of Florida have identified specific gut bacteria that promoted protein misfolding throughout the body, as well as others that were protective.