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Daniela Ernst

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Articles 6
Citations 236
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Recent Articles
1.
Alecu I, Tedeschi A, Behler N, Wunderling K, Lamberz C, Lauterbach M, et al.
J Lipid Res . 2016 Nov; 58(1):42-59. PMID: 27881717
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...
2.
Zhakupova A, Debeuf N, Krols M, Toussaint W, Vanhoutte L, Alecu I, et al.
FASEB J . 2016 Sep; 30(12):4289-4300. PMID: 27645259
ORMDL proteins are believed to be negative regulators of serine palmitoyltransferase (SPT), which catalyzes the first and rate limiting step in sphingolipid (SL) de novo synthesis. Several single-nucleotide polymorphisms (SNPs)...
3.
Kramer R, Bielawski J, Kistner-Griffin E, Othman A, Alecu I, Ernst D, et al.
FASEB J . 2015 Jul; 29(11):4461-72. PMID: 26198449
Peripheral neuropathy is a major dose-limiting side effect of paclitaxel and cisplatin chemotherapy. In the current study, we tested the involvement of a novel class of neurotoxic sphingolipids, the 1-deoxysphingolipids....
4.
Ernst D, Murphy S, Sathiyanadan K, Wei Y, Othman A, Laura M, et al.
Neuromolecular Med . 2015 Jan; 17(1):47-57. PMID: 25567748
1-Deoxysphingolipids (1-deoxySL) are atypical sphingolipids that are formed by the enzyme serine palmitoyltransferase (SPT) due to a promiscuous use of L-alanine over its canonical substrate L-serine. Several mutations in SPT...
5.
Murphy S, Ernst D, Wei Y, Laura M, Liu Y, Polke J, et al.
Neurology . 2013 May; 80(23):2106-11. PMID: 23658386
Objective: To describe the clinical and neurophysiologic phenotype of a family with hereditary sensory and autonomic neuropathy type 1 (HSANI) due to a novel mutation in SPTLC2 and to characterize...
6.
Hornemann T, Penno A, Rutti M, Ernst D, Kivrak-Pfiffner F, Rohrer L, et al.
J Biol Chem . 2009 Aug; 284(39):26322-30. PMID: 19648650
The enzyme serine palmitoyltransferase (SPT) catalyzes the rate-limiting step in the de novo synthesis of sphingolipids. Previously the mammalian SPT was described as a heterodimer composed of two subunits, SPTLC1...