Supplementary Materials

Supplementary Material for:

Ectopic calcification in pseudoxanthoma elasticum responds to inhibition of tissue-nonspecific alkaline phosphatase

Shira G. Ziegler,* Carlos R. Ferreira, Elena Gallo MacFarlane, Ryan C. Riddle, Ryan E. Tomlinson, Emily Y. Chew, Ludovic Martin, Chen-Ting Ma, Eduard Sergienko, Anthony B. Pinkerton, José Luis Millán, William A. Gahl, Harry C. Dietz*

*Corresponding author. Email: sgziegler{at}jhmi.edu (S.G.Z.); hdietz{at}jhmi.edu (H.C.D.)

Published 7 June 2017, Sci. Transl. Med. 9, eaal1669 (2017)
DOI: 10.1126/scitranslmed.aal1669

This PDF file includes:

  • Materials and Methods
  • Fig. S1. Location of calcification in the fibrous capsule surrounding the vibrissae.
  • Fig. S2. Demonstration of genetic interaction between Abcc6 and Nt5e mice when aged to 1 year.
  • Fig. S3. Demonstration of efficient liver-specific deletion of Abcc6 in mice.
  • Fig. S4. Primary dermal fibroblasts derived from patients with biallelic mutations in ABCC6 show TNAP-dependent in vitro calcification.
  • Fig. S5. TNAP inhibition does not alter circulating PPi concentration in mice.
  • Fig. S6. TNAP inhibition had no negative effects on bone microarchitecture or mineralization in a PXE mouse model.
  • Fig. S7. TNAP inhibition prevents progression of established calcification in a PXE mouse model.
  • Table S1. List of patient mutations in ABCC6, ENPP1, and NT5E.
  • Table S2. Number of calcified mice at 20 weeks and 1 year of age.
  • Table S3. Cortical bone microarchitecture.
  • Table S4. Cortical bone strength.
  • References (4651)

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Other Supplementary Material for this manuscript includes the following:

  • Table S5 (Microsoft Excel format). Individual-level data.

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