Spinal manifestations of CLN1 disease start during the early postnatal period.

ABSTRACT:

Aim

To understand the progression of CLN1 disease and develop effective therapies we need to characterize early sites of pathology. Therefore, we performed a comprehensive evaluation of the nature and timing of early CLN1 disease pathology in the spinal cord, which appears especially vulnerable, and how this may affect behaviour.

Methods

We measured the spinal volume and neuronal number, and quantified glial activation, lymphocyte infiltration and oligodendrocyte maturation, as well as cytokine profile analysis during the early stages of pathology in Ppt1-deficient (Ppt1^-/- ) mouse spinal cords. We then performed quantitative gait analysis and open-field behaviour tests to investigate the behavioural correlates during this period.

Results

We detected signif

SUBMITTER: Nelvagal HR 

PROVIDER: S-EPMC7867600 | biostudies-literature | 2021 Feb

REPOSITORIES: biostudies-literature