Richard W D Welford
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Explore the profile of Richard W D Welford including associated specialties, affiliations and a list of published articles.
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20
Citations
532
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Recent Articles
1.
Schidlitzki A, Stanojlovic M, Fournier C, Kaufer C, Feja M, Gericke B, et al.
Mov Disord
. 2023 Apr;
38(6):1044-1055.
PMID: 37050861
Background: Venglustat is a brain-penetrant, small molecule inhibitor of glucosylceramide synthase used in clinical testing for treatment of Parkinson's disease (PD). Despite beneficial effects in certain cellular and rodent models,...
2.
Welford R, Farine H, Steiner M, Garzotti M, Dobrenis K, Sievers C, et al.
Mol Genet Metab Rep
. 2022 Mar;
30:100843.
PMID: 35242574
GM2 and GM1 gangliosidoses are genetic, neurodegenerative lysosomal sphingolipid storage disorders. The earlier the age of onset, the more severe the clinical presentation and progression, with infantile, juvenile and late-onset...
3.
Schafer A, Haenig B, Erupathil J, Strickner P, Sabato D, Welford R, et al.
Oncogene
. 2021 Jan;
40(9):1659-1673.
PMID: 33500549
The clinical benefit of MAPK pathway inhibition in melanoma patients carrying BRAF mutations is temporal. After the initial response to treatment, the majority of tumors will develop resistance and patients...
4.
Gehin M, Melchior M, Welford R, Sidharta P, Dingemanse J
Clin Transl Sci
. 2020 Nov;
14(2):558-567.
PMID: 33142037
In this first-in-human study, the tolerability, pharmacokinetics (PK), and pharmacodynamics (PD) of single and multiple oral doses of sinbaglustat, a dual inhibitor of glucosylceramide synthase (GCS) and non-lysosomal glucosyl ceramidase...
5.
Schmidt A, Farine H, Keller M, Sebastian A, Kozera L, Welford R, et al.
J Proteome Res
. 2020 Sep;
19(10):4196-4209.
PMID: 32870689
One of the most important advantages of mass spectrometry is the ability to quantify proteins and their modifications in parallel to obtain a holistic picture of the protein of interest....
6.
Sieber P, Schafer A, Lieberherr R, Le Goff F, Stritt M, Welford R, et al.
PLoS One
. 2018 Nov;
13(11):e0207872.
PMID: 30485339
Pathological features of pulmonary fibrosis include accumulation of myofibroblasts and increased extracellular matrix (ECM) deposition in lung tissue. Contractile α-smooth muscle actin (α-SMA)-expressing myofibroblasts that produce and secrete ECM are...
7.
Gehin M, Welford R, Garzotti M, Vercauteren M, Groenen P, Nayler O, et al.
Clin Pharmacol Ther
. 2018 Apr;
104(6):1260-1267.
PMID: 29663345
Serotonin (5-HT) is synthesized from dietary tryptophan (Trp) and plays an important role in numerous diseases of the central nervous system and periphery. Stable isotope tracers enable safe monitoring of...
8.
Guerard N, Oder D, Nordbeck P, Zwingelstein C, Morand O, Welford R, et al.
Clin Pharmacol Ther
. 2017 Jul;
103(4):703-711.
PMID: 28699267
Lucerastat is a glucosylceramide synthase inhibitor aimed at reducing production of glycosphingolipids (GSLs), including those accumulating in Fabry disease. The safety, tolerability, pharmacodynamics, and pharmacokinetics of oral lucerastat were evaluated...
9.
Welford R, Vercauteren M, Trebaul A, Cattaneo C, Eckert D, Garzotti M, et al.
Sci Rep
. 2016 Jul;
6:30059.
PMID: 27444653
The biogenic amine serotonin (5-HT) is a multi-faceted hormone that is synthesized from dietary tryptophan with the rate limiting step being catalyzed by the enzyme tryptophan hydroxylase (TPH). The therapeutic...
10.
Vanier M, Gissen P, Bauer P, Coll M, Burlina A, Hendriksz C, et al.
Mol Genet Metab
. 2016 Jun;
118(4):244-54.
PMID: 27339554
Niemann-Pick disease type C (NP-C) is a neurovisceral lysosomal cholesterol trafficking and lipid storage disorder caused by mutations in one of the two genes, NPC1 or NPC2. Diagnosis has often...