Joana Carvalho, PhD, managing science editor —

Chemical compounds similar to flupirtine can effectively prevent cell death in Batten disease patient-derived cell lines, a study reports. These findings provide the first preclinical evidence pointing toward the therapeutic effects of such compounds and could be the first step in establishing potential treatments for Batten disease in both…

New Zealand researchers used computed tomography (CT) to measure and monitor brain alterations in sheep models of Batten disease, a study reports. Data revealed that brain atrophy starts in specific regions of the brain, called occipital lobes, spreads to the whole cortex, and eventually leads to a severe reduction…

Two types of nerve cells — microglia and astrocytes — are dysfunctional and may contribute to neurodegeneration associated with different types of neuronal ceroid lipofuscinoses (NCLs, aka Batten disease), according to a new mouse study. However, the reasons behind these cells’ dysfunction are distinct in infantile NCL (…

Agitation and involuntary motor activity, which normally occurs in individuals who suffered traumatic brain injuries, also happens in patients with juvenile neuronal ceroid lipofuscinosis (JNCL, also known as Batten disease), according to researchers. The study, “Paroxysmal sympathetic hyperactivity in Juvenile neuronal ceroid lipofuscinosis (Batten disease),” was published in Autonomic…

Researchers have described how the CLN5 protein — the mutation of which underlies a type of Batten disease — turns into a mature soluble protein, information they hope can be used to identify new therapeutic strategies for the disease. The study, “CLN5 is cleaved by members…

Researchers have identified a previously unknown function for the CLN3 protein, whose defective form underlies juvenile Batten disease. The study, “Role of the Lysosomal Membrane Protein, CLN3, in the Regulation of Cathepsin D Activity,” was published in the Journal of Cellular Biochemistry. Neuronal ceroid lipofuscinoses (NCLs),…