Focus on Hydrocephalus

Hydrocephalus affects one in 1,000 children, with most of the cases require multiple brain surgeries. June Goto, PhD, collaborating in the Mangano lab, studies mouse and rat models of pediatric hydrocephalus looking for potential medicine and new diagnostic tools for this condition. The lab, along with collaborators Kenneth Campbell, PhD, and Rolf Stottmann, PhD, focuses on primary and motile ciliary signaling essential for proper development of neural cells and their functions. They identified a novel gene mutation affecting ciliogenesis in specific subtypes of developing neural cells leading to communicating hydrocephalus. Also, in collaboration with the Pediatric Neuroimaging Research Center, and Weihong Yuan, PhD, the lab investigate molecular and cellular features of white matter damage in hydrocephalus models.

Studying our brains to improve breathing

Dr. Steven Crone, PhD, heads a laboratory studying how neural circuits in our brain and spinal cord control movements such as breathing and locomotion, and how disease and injury affects them. Dr. Crone’s laboratory uses unique mouse models, and a powerful physiological monitoring system, to find new ways to improve breathing in amyotrophic lateral sclerosis (ALS), spinal muscular atrophy (SMA), and spinal cord injury. In the past year, his laboratory has demonstrated the use of muscles, other than the diaphragm, called accessory respiratory muscles, during early stages of disease in a mouse model of ALS. However, these muscles stop being used for breathing at late stages of disease, despite the fact that the muscles are functional and used for other behaviors, such as grooming. Further, they have identified a neuron class in the spinal cord and brainstem that controls the activity of these muscles and shown that these neurons degenerate in ALS model mice. Research is currently underway to develop therapies to repair or replace these neurons and assess the impact on breathing, motor function, and survival. The goal of this work is to prevent ventilator dependence, and improve the duration and quality of life of patients with neuromuscular disorders or spinal cord injury.