Ponatinib-d is intended for use as an internal standard for the quantification of ponatinib by GC- or LC-MS. Ponatinib is an orally bioavailable Bcr-Abl tyrosine kinase inhibitor (IC = 0.37 nM). It inhibits the tyrosine kinase inhibitor-resistant mutant Bcr-Abl (IC = 2 nM), as well as Bcr-Abl, Bcr-Abl, Bcr-Abl, and Bcr-Abl mutants (ICs = 0.44, 0.3, 0.3, and 0.34 nM, respectively). It is selective for Bcr-Abl and these mutants over the insulin receptor, IGF-1R, Aurora A kinase, and Cdk2/cyclin E but does inhibit the receptor tyrosine kinases c-Src, VEGF receptor 2 (VEGFR2), FGFR1, and PDGFRα (ICs = 5.4, 1.5, 2.2, and 1.1 nM, respectively). Ponatinib inhibits proliferation of Ba/F3 cells expressing native (IC = 0.5 nM) or mutant Bcr-Abl (ICs = 0.5-36 nM) and induces apoptosis. It reduces tumor growth in a Ba/F3 Bcr-Abl mouse xenograft model when administered at doses ranging from 10 to 30 mg/kg. Formulations containing ponatinib have been used in the treatment of chronic-, accelerated-, or blast-phase chronic myeloid leukemia (CML), T315I-positive CML, or T315I-positive Philadelphia-chromosome positive acute lymphoblastic leukemia (Ph+ ALL).