Abnormal Development of Neural Stem Cell Niche in the Dentate Gyrus of Menkes Disease.

ABSTRACT:

Background and objectives

Menkes disease (MNK) is a rare X-linked recessive disease, caused by mutations in the copper transporting ATP7A gene that is required for copper homeostasis. MNK patients experience various clinical symptoms including neurological defects that are closely related to the prognosis of MNK patients. Neural stem cells (NSCs) in the hippocampal dentate gyrus (DG) produce new neurons throughout life, and defects in DG neurogenesis are often correlated with cognitive and behavioral problems. However, neurodevelopmental defects in the DG during postnatal period in MNK have not been understood yet.

Methods and results

Mottled-brindled (Mo^Br/y) mice (MNK mice) and littermate controls were used in this study. In vivo microCT imaging and immun

SUBMITTER: Cho SK 

PROVIDER: S-EPMC9396019 | biostudies-literature | 2022 Aug

REPOSITORIES: biostudies-literature