High resolution 1H NMR-based metabolomics indicates a neurotransmitter cycling deficit in cerebral tissue from a mouse model of Batten disease
MR Pears, JD Cooper, HM Mitchison… - Journal of Biological …, 2005 - ASBMB
The neuronal ceroid lipofuscinoses (NCLs) constitute a range of progressive neurological
disorders primarily affecting children. Although six of the causative genes have been
characterized, the underlying disease pathogenesis for this family of disorders is unknown.
Using a metabolomics approach based on high resolution 1 H NMR spectroscopy of the
cortex, cerebellum, and remaining regions of the brain in conjunction with statistical pattern
recognition, we report metabolic deficits associated with juvenile NCL in a Cln3 knock-out …
disorders primarily affecting children. Although six of the causative genes have been
characterized, the underlying disease pathogenesis for this family of disorders is unknown.
Using a metabolomics approach based on high resolution 1 H NMR spectroscopy of the
cortex, cerebellum, and remaining regions of the brain in conjunction with statistical pattern
recognition, we report metabolic deficits associated with juvenile NCL in a Cln3 knock-out …
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