[HTML][HTML] Localization of 1-deoxysphingolipids to mitochondria induces mitochondrial dysfunction
1-Deoxysphingolipids (deoxySLs) are atypical sphingolipids that are elevated in the plasma
of patients with type 2 diabetes and hereditary sensory and autonomic neuropathy type 1
(HSAN1). Clinically, diabetic neuropathy and HSAN1 are very similar, suggesting the
involvement of deoxySLs in the pathology of both diseases. However, very little is known
about the biology of these lipids and the underlying pathomechanism. We synthesized an
alkyne analog of 1-deoxysphinganine (doxSA), the metabolic precursor of all deoxySLs, to …
of patients with type 2 diabetes and hereditary sensory and autonomic neuropathy type 1
(HSAN1). Clinically, diabetic neuropathy and HSAN1 are very similar, suggesting the
involvement of deoxySLs in the pathology of both diseases. However, very little is known
about the biology of these lipids and the underlying pathomechanism. We synthesized an
alkyne analog of 1-deoxysphinganine (doxSA), the metabolic precursor of all deoxySLs, to …