Analyzing the Therapeutic Efficacy of Bis-Choline-Tetrathiomolybdate in the

ALXN-1840 Atp7b D-penicillamine WTX101 Wilson’s disease bis-choline-tetrathiomolybdate copper laser ablation inductively coupled plasma mass spectrometry therapy trientine

Journal

Biomedicines
ISSN: 2227-9059
Titre abrégé: Biomedicines
Pays: Switzerland
ID NLM: 101691304

Informations de publication

Date de publication:
08 Dec 2021
Historique:
received: 04 11 2021
revised: 05 12 2021
accepted: 06 12 2021
entrez: 24 12 2021
pubmed: 25 12 2021
medline: 25 12 2021
Statut: epublish

Résumé

Bis-choline-tetrathiomolybdate, introduced as WTX101 (now known as ALXN1840), is a first-in-class copper-protein-binding agent for oral therapy of Wilson's disease. In contrast to other decoppering agents such as trientine or D-penicillamine it acts by forming a tripartite complex with copper and albumin, thereby detoxifying excess liver and blood copper through biliary excretion. Preclinical animal experimentation with this drug was typically done with the alternative ammonium salt of tetrathiomolybdate, which is expected to have identical properties in terms of copper binding. Here, we comparatively analyzed the therapeutic efficacy of ALXN1840, D-penicillamine and trientine in lowering hepatic copper content in

Identifiants

pubmed: 34944677
pii: biomedicines9121861
doi: 10.3390/biomedicines9121861
pmc: PMC8698685
pii:
doi:

Types de publication

Journal Article

Langues

eng

Subventions

Organisme : Deutsche Forschungsgemeinschaft
ID : WE 2554/13-1, WE 2554/15-1, WE 2554/17-1

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Auteurs

Philipp Kim (P)

Institute of Molecular Pathobiochemistry, Experimental Gene Therapy and Clinical Chemistry (IFMPEGKC), RWTH University Hospital Aachen, D-52074 Aachen, Germany.

Chengcheng Christine Zhang (CC)

Department of Internal Medicine IV, Heidelberg University Hospital, D-69120 Heidelberg, Germany.

Sven Thoröe-Boveleth (S)

Institute for Occupational, Social and Environmental Medicine, RWTH Aachen University, D-52074 Aachen, Germany.

Eva Miriam Buhl (EM)

Electron Microscopy Facility, Institute of Pathology, RWTH Aachen University Hospital, D-52074 Aachen, Germany.

Sabine Weiskirchen (S)

Institute of Molecular Pathobiochemistry, Experimental Gene Therapy and Clinical Chemistry (IFMPEGKC), RWTH University Hospital Aachen, D-52074 Aachen, Germany.

Wolfgang Stremmel (W)

Medical Center Baden-Baden, D-76530 Baden-Baden, Germany.

Uta Merle (U)

Department of Internal Medicine IV, Heidelberg University Hospital, D-69120 Heidelberg, Germany.

Ralf Weiskirchen (R)

Institute of Molecular Pathobiochemistry, Experimental Gene Therapy and Clinical Chemistry (IFMPEGKC), RWTH University Hospital Aachen, D-52074 Aachen, Germany.

Classifications MeSH