Online supplement to: miRNA-10a Regulation of Proinflammatory Mediators: An Important Component of Untreated Juvenile Dermatomyositis. doi:10.3899/jrheum.141474

ONLINE SUPPLEMENTARY DATA

Supplementary Table 1. Demographics of 15 untreated children with JDM and 5 healthy controls. Five healthy pediatric controls and 15 children with JDM (characterized with respect, age at biopsy, sex, race). In addition, the JDM were categorized concerning the DUD at biopsy and their myositis-specific antibody status. The muscle was tested by microRNA array profiling, qRT-PCR validation for miR-10a and miR-10b, and measurement of IL-6 and VCAM-1 expression.

Case Sex Race Diagnosis Age at Muscle DUD, Mos Myositis-specific No. Biopsy, Yrs Antibodies Present

1 Female White JDM 2.43 4.53 p155/140, Mi-2 indeterminate

2 Male White JDM 2.56 1.08 none 3 Female Asian JDM 3.22 1.68 none 4 Male White JDM 3.43 12.81 none 5 Female White JDM 3.59 32.00 not tested 6 Male White JDM 4.14 2.83 p155/140 7 Female White JDM 5.84 2.89 Mi-2 indeterminate 8 Female White JDM 6.27 1.22 MJ indeterminate 9 Male White JDM 6.39 3.68 p155/140 10 Female White JDM 7.00 1.71 none 11 Female Hispanic Probable JDM 7.92 1.08 MJ indeterminate 12 Male White JDM 8.02 1.12 none 13 Female White JDM 8.32 15.84 p155/140, Mi-2 indeterminate

14 Female White JDM 9.69 3.65 Mi-2 15 Male Hispanic JDM 13.42 11.10 none 16 Male White Healthy 5.28 N/A not tested Control 17 Male White Healthy 12.43 N/A not tested Control 18 Male White Healthy 12.79 N/A not tested Control 19 Female White Healthy 14.70 N/A not tested Control 20 Female White Healthy 16.26 N/A not tested Control

JDM: juvenile dermatomyositis; DUD: duration of untreated disease; qRT-PCR: quantitative real- time PCR; IL-6: interleukin 6; VCAM-1: vascular molecule 1; N/A: not applicable.

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Online supplement to: miRNA-10a Regulation of Proinflammatory Mediators: An Important Component of Untreated Juvenile Dermatomyositis. doi:10.3899/jrheum.141474

Supplementary Table 2. miRNA-10 target gene list. Targetscan was performed and identified 178 miR-10 target . Search of our published profile data from 22 untreated children with JDM1 revealed that 32 of the 178 miR-10 target genes were significantly changed more than 1.5-fold with p < 0.05. MAP3K7 and ß-TRC are included on this list with a 1.67- and 1.85-fold increase, respectively; they interact with miR-10a and -10b to control NF-κB regulated proinflammatory cytokine production. Relative expression level in controls was “1”.

Gene Symbol Fold Change ABCG1 3.62 ARHGAP12 2.4 PALM2 2.32 ZFP36L1 2.05 SLC25A11 2.01 SCARB2 1.96 ACTG1 1.94 MAPRE1 1.93 SHC1 1.9 BAZ2B 1.87 BTRC 1.85 SETD5 1.7 MAP3K7 1.67 CUGBP2 1.63 NFASC 1.55 POPDC2 1.54 CEP350 1.54 NF1 1.5 LPHN1 –1.52 KBTBD11 –1.59 PAP2B –1.59 BICD2 –1.66 GALNT1 –1.66 NCOR2 –1.73 CTDSPL –1.83 PPARA –1.85 ELAVL3 –2.14 CAMK2G –2.2 HDAC4 –2.2 SOBP –2.3 ANK3 –2.37 FHL3 –2.54

JDM: juvenile dermatomyositis; MAP3K7: mitogen-activated kinase kinase kinase 7; ß- TRC: ß transducin repeat containing E3 ubiquitin protein ligase; NF-κB: nuclear factor-κB

REFERENCES

1. Zarif MN, Soleimani M, Abolghasemi H, Amirizade N, Arefian E, Rahimian A. Megakaryocytic differentiation of CD133+ hematopoietic stem cells by down-regulation of microRNA-10a. Hematology 2013;18:93-100.

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