Year: 2023
Type: article
Abstract: Mucopolysaccharidosis type I (MPS I) is an inherited lysosomal disorder that causes syndromes characterized by physiological dysfunction in many organs and tissues. Despite the recognizable morphological and behavioral deficits associated with MPS I, neither the underlying alterations in functional neural connectivity nor its restoration following gene therapy have been shown. By employing high-resolution resting-state fMRI (rs-fMRI), we found significant reductions in functional neural connectivity in the limbic areas of the brain that play key roles in learning and memory in MPS I mice, and that adeno-associated virus (AAV)-mediated gene therapy can reestablish most brain connectivity. Using logistic regression in MPS I and treated animals, we identified functional networks with the most alterations. The rs-fMRI and statistical methods should be translatable into clinical evaluation of humans with neurological disorders.
Source: Scientific Reports
Authors: Wei Zhu, Li Ou, Lin Zhang, Isaac H. Clark, Ying Zhang, Xiao-Hong Zhu, Chester B. Whitley, Perry B. Hackett, Walter C. Low, Wei Chen
Language: English
Subfield: Cognitive Neuroscience
Field: Neuroscience
Domain: Life Sciences
Open Access status: gold
Estimated APC paid: $2,390
Awards: Advancing simultaneous fMRI-multiphoton imaging technique to study brain function and connectivity across different scales at ultrahigh field, Breaking Spatiotemporal Barriers of MR Imaging Technologies to Study Human Brain Function and Neuroenergetics, Integrated fMRI Methods to Study Neurophysiology and Circuit Dynamics at Laminar and Columnar Level, G9000308991, Establishing translational neuroimaging tools for quantitative assessment of energy metabolism and metabolic reprogramming in healthy and diseased human brain at 7T, Technology to Realize the Full Potential of UHF MRI
OpenAlex ID: W4385606616