D. Holmes
Oct 21, 2014
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Nature Reviews Endocrinology
Abstract
development of motor neurons and for their maintenance in adult life; however, until now, no endogenous ligand capable of regulating the survival of motor neurons has been identified,” explains study investigator William Griffiths. The researchers used liquid chromatography–electrospray ionization– mass spectrometry to analyse the cholestenoic acid profile of cerebrospinal fluid (CSF) and plasma isolated from patients with two neurological diseases associated with motor neuron degeneration: cerebrotendinous xanthomatosis (CTX); and hereditary spastic paresis type 5 (SPG5). Patients with CTX or SPG5 had reduced CSF and plasma levels of 3β,7α-dihydroxycholest-5-en26-oic acid (3β,7α-diHCA); patients with SPG5 additionally had elevated levels of 3β-hydroxycholest-5-en26-oic acid (3β-HCA). Functional analyses of these cholestenoic acids in mice revealed that 3β,7α-diHCA promoted motor neuron survival in NEUROENDOCRINOLOGY