Patricia Inácio, PhD,  science writer—

Researchers found, in a new mouse model for Batten disease, that defective synapses in specific brain regions may be the cause of disease symptoms. The study “Defective synaptic transmission causes disease signs in a mouse model of juvenile neuronal ceroid lipofuscinosis,” was published in the journal eLIFE. Batten…

Glial cell function is impaired in the juvenile form of Batten disease, leading to nerve cell loss, a mouse study has found. The study, “Glial cells are functionally impaired in juvenile neuronal ceroid lipofuscinosis and detrimental to neurons,” was appeared in the journal Acta Neuropathologica Communications. Glial cells,…

Early photosensitivity may be a hallmark of ceroid lipofuscinosis type 2 (CLN2) disease, according to the results of a small study. The study “Photosensitivity is an early marker of neuronal ceroid lipofuscinosis type 2 disease” was published in the journal Epilepsia. The neuronal ceroid lipofuscinoses (NCLs), or Batten disease, are a heterogeneous group of lysosomal storage disorders that…

Current therapeutics, including gene therapy, that are based on delivery of TTP1 protein directly into the central nervous system of patients with Batten disease may not be enough to prevent all the symptoms associated with this condition. Using a canine model for the disease, researchers showed that this approach did…