Proton magnetic resonance spectroscopy detects cerebral metabolic derangement in a mouse model of brain coenzyme a deficiency.

ABSTRACT:

Background

Pantothenate kinase (PANK) is the first and rate-controlling enzymatic step in the only pathway for cellular coenzyme A (CoA) biosynthesis. PANK-associated neurodegeneration (PKAN), formerly known as Hallervorden-Spatz disease, is a rare, life-threatening neurologic disorder that affects the CNS and arises from mutations in the human PANK2 gene. Pantazines, a class of small molecules containing the pantazine moiety, yield promising therapeutic effects in an animal model of brain CoA deficiency. A reliable technique to identify the neurometabolic effects of PANK dysfunction and to monitor therapeutic responses is needed.

Methods

We applied ¹H magnetic resonance spectroscopy as a noninvasive technique to evaluate the therapeutic effects of the newly devel

SUBMITTER: Li Y 

PROVIDER: S-EPMC8867880 | biostudies-literature | 2022 Feb

REPOSITORIES: biostudies-literature