Identification of Rigosertib for the Treatment of Recessive Dystrophic Epidermolysis Bullosa-Associated Squamous Cell Carcinoma.


Journal

Clinical cancer research : an official journal of the American Association for Cancer Research
ISSN: 1557-3265
Titre abrégé: Clin Cancer Res
Pays: United States
ID NLM: 9502500

Informations de publication

Date de publication:
01 06 2019
Historique:
received: 15 08 2018
revised: 11 01 2019
accepted: 21 02 2019
pubmed: 9 3 2019
medline: 7 7 2020
entrez: 9 3 2019
Statut: ppublish

Résumé

Squamous cell carcinoma (SCC) of the skin is the leading cause of death in patients with the severe generalized form of the genetic disease recessive dystrophic epidermolysis bullosa (RDEB). Although emerging data are identifying why patients suffer this fatal complication, therapies for treatment of RDEB SCC are in urgent need. ON-01910 (or rigosertib) exhibited significant specificity for RDEB SCC: in culture rigosertib induced apoptosis in 10 of 10 RDEB SCC keratinocyte populations while only slowing the growth of normal primary skin cells at doses 2 orders of magnitude higher. Furthermore, rigosertib significantly inhibited the growth of two RDEB SCC in murine xenograft studies with no apparent toxicity. Mechanistically, rigosertib has been shown to inhibit multiple signaling pathways. Comparison of PLK1 siRNA with MEK inhibition, AKT inhibition, and the microtubule-disrupting agent vinblastine in RDEB SCC shows that only PLK1 reduction exhibits a similar sensitivity profile to rigosertib. These data support a "first in RDEB" phase II clinical trial of rigosertib to assess tumor targeting in patients with late stage, metastatic, and/or unresectable SCC.

Identifiants

pubmed: 30846478
pii: 1078-0432.CCR-18-2661
doi: 10.1158/1078-0432.CCR-18-2661
pmc: PMC8185613
mid: NIHMS1701557
doi:

Substances chimiques

Antineoplastic Agents 0
Cell Cycle Proteins 0
Proto-Oncogene Proteins 0
RNA, Messenger 0
RNA, Small Interfering 0
Sulfones 0
ON 01910 67DOW7F9GL
Protein Serine-Threonine Kinases EC 2.7.11.1
Glycine TE7660XO1C

Types de publication

Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, Non-P.H.S.

Langues

eng

Sous-ensembles de citation

IM

Pagination

3384-3391

Subventions

Organisme : NCI NIH HHS
ID : P30 CA056036
Pays : United States

Informations de copyright

©2019 American Association for Cancer Research.

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Auteurs

Velina S Atanasova (VS)

Department of Dermatology and Cutaneous Biology, Thomas Jefferson University, Philadelphia, Pennsylvania.

Celine Pourreyron (C)

Division of Cellular Medicine, University of Dundee, Dundee, United Kingdom.

Mehdi Farshchian (M)

Department of Dermatology and Cutaneous Biology, Thomas Jefferson University, Philadelphia, Pennsylvania.

Michael Lawler (M)

Department of Dermatology and Cutaneous Biology, Thomas Jefferson University, Philadelphia, Pennsylvania.

Christian A Brown (CA)

Department of Dermatology and Cutaneous Biology, Thomas Jefferson University, Philadelphia, Pennsylvania.

Stephen A Watt (SA)

Division of Cellular Medicine, University of Dundee, Dundee, United Kingdom.

Sheila Wright (S)

Division of Cellular Medicine, University of Dundee, Dundee, United Kingdom.

Michael Warkala (M)

Department of Dermatology and Cutaneous Biology, Thomas Jefferson University, Philadelphia, Pennsylvania.

Christina Guttmann-Gruber (C)

EB House Austria, Research Program for Molecular Therapy of Genodermatoses, Department of Dermatology, University Hospital of the Paracelsus Medical University Salzburg, Austria.

Josefina Piñón Hofbauer (JP)

EB House Austria, Research Program for Molecular Therapy of Genodermatoses, Department of Dermatology, University Hospital of the Paracelsus Medical University Salzburg, Austria.

Ignacia Fuentes (I)

Fundación DEBRA Chile, Santiago, Chile.
Centro de Genética y Genómica, Facultad de Medicina Clínica Alemana, Universidad del Desarrollo, Santiago, Chile.

Marco Prisco (M)

Department of Dermatology and Cutaneous Biology, Thomas Jefferson University, Philadelphia, Pennsylvania.

Elham Rashidghamat (E)

St. John's Institute of Dermatology, King's College London (Guy's Campus), London, United Kingdom.

Cristina Has (C)

Department of Dermatology, Medical Center-University of Freiburg, Faculty of Medicine, Freiburg, Germany.

Julio C Salas-Alanis (JC)

Instituto Dearmatologico de Jalisco, Guadalajara, Mexico.

Francis Palisson (F)

Fundación DEBRA Chile, Santiago, Chile.
Facultad de Medicina Clínica Alemana, Universidad del Desarrollo, Santiago, Chile.

Alain Hovnanian (A)

INSERM UMR 1163, Paris, France.
Imagine Institute, Paris, France.

John A McGrath (JA)

St. John's Institute of Dermatology, King's College London (Guy's Campus), London, United Kingdom.

Jemima E Mellerio (JE)

St. John's Institute of Dermatology, King's College London (Guy's Campus), London, United Kingdom.

Johann W Bauer (JW)

EB House Austria, Research Program for Molecular Therapy of Genodermatoses, Department of Dermatology, University Hospital of the Paracelsus Medical University Salzburg, Austria.

Andrew P South (AP)

Department of Dermatology and Cutaneous Biology, Thomas Jefferson University, Philadelphia, Pennsylvania. Andrew.south@Jefferson.edu.

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