Interleukin 1 Receptor-Like 1 Protein (ST2) is a Potential Biomarker for Cardiomyopathy in Duchenne Muscular Dystrophy

On Behalf Of The Cinrg Investigators

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

Duchenne muscular dystrophy (DMD) is a rare, fatal X-linked disorder characterized by the lack of dystrophin, a key sarcolemma muscle protein. Cardiac failure is a significant cause of death in DMD subjects. The purpose of our research was to identify potential cardiac serum biomarkers associated with DMD cardiomyopathy. This is an observational, case-controlled study using subjects from the CINRG DMD natural history study with cardiomyopathy (ejection fraction (EF) <55%; shortening fraction (SF) <28%), subjects without cardiomyopathy (EF ≥ 55%; SF ≥ 28%) compared to normal healthy volunteer subjects. The DMD with cardiomyopathy group had significantly lower average EF and SF (EF = 45 ± 10/SF = 25 ± 2%) than the DMD without cardiomyopathy group (EF = 58 ± 5% and SF = 32 ± 3%; p < 0.01). Among a selected set of potential biomarkers for cardiomyopathy (MMP9, BNP, GAL3, CRP, LEP, TNC, TLR4 and ST2) we validated ST2 as significantly elevated in the serum of DMD cardiomyopathy group (35,798 ± 4884 pg/mL) compared to normal controls (9940 ± 2680 pg/mL; p < 0.01; n = 6). Matrix metallopeptidase 9 (MMP9) levels were found significantly increased in both DMD groups compared to controls (p < 0.01). No significant differences were seen in BNP, GAL3, CRP, LEP, TNC or TLR4 levels. Increased ST2 levels were found in serum of DMD subjects compared to healthy volunteers and further elevated in DMD subjects with cardiomyopathy. Future studies correlating cardiomyopathy with ST2 levels may allow for improved non-invasive monitoring of cardiac disease in DMD subjects.

Original languageEnglish (US)
Pages (from-to)1-7
Number of pages7
JournalPediatric Cardiology
DOIs
StateAccepted/In press - Aug 18 2017
Externally publishedYes

Fingerprint

Duchenne Muscular Dystrophy
Cardiomyopathies
Biomarkers
Healthy Volunteers
Metalloproteases
Interleukin-1 Receptor-Like 1 Protein
Serum
Sarcolemma
Dystrophin
Muscle Proteins
Natural History
Cause of Death
Heart Diseases
Heart Failure

Keywords

  • Biomarkers
  • Cardiomyopathy
  • Duchenne muscular dystrophy
  • ST2

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Cardiology and Cardiovascular Medicine

Cite this

Interleukin 1 Receptor-Like 1 Protein (ST2) is a Potential Biomarker for Cardiomyopathy in Duchenne Muscular Dystrophy. / On Behalf Of The Cinrg Investigators.

In: Pediatric Cardiology, 18.08.2017, p. 1-7.

Research output: Contribution to journalArticle

@article{7dafd08ddcd643508eceac4259a07b7d,
title = "Interleukin 1 Receptor-Like 1 Protein (ST2) is a Potential Biomarker for Cardiomyopathy in Duchenne Muscular Dystrophy",
abstract = "Duchenne muscular dystrophy (DMD) is a rare, fatal X-linked disorder characterized by the lack of dystrophin, a key sarcolemma muscle protein. Cardiac failure is a significant cause of death in DMD subjects. The purpose of our research was to identify potential cardiac serum biomarkers associated with DMD cardiomyopathy. This is an observational, case-controlled study using subjects from the CINRG DMD natural history study with cardiomyopathy (ejection fraction (EF) <55{\%}; shortening fraction (SF) <28{\%}), subjects without cardiomyopathy (EF ≥ 55{\%}; SF ≥ 28{\%}) compared to normal healthy volunteer subjects. The DMD with cardiomyopathy group had significantly lower average EF and SF (EF = 45 ± 10/SF = 25 ± 2{\%}) than the DMD without cardiomyopathy group (EF = 58 ± 5{\%} and SF = 32 ± 3{\%}; p < 0.01). Among a selected set of potential biomarkers for cardiomyopathy (MMP9, BNP, GAL3, CRP, LEP, TNC, TLR4 and ST2) we validated ST2 as significantly elevated in the serum of DMD cardiomyopathy group (35,798 ± 4884 pg/mL) compared to normal controls (9940 ± 2680 pg/mL; p < 0.01; n = 6). Matrix metallopeptidase 9 (MMP9) levels were found significantly increased in both DMD groups compared to controls (p < 0.01). No significant differences were seen in BNP, GAL3, CRP, LEP, TNC or TLR4 levels. Increased ST2 levels were found in serum of DMD subjects compared to healthy volunteers and further elevated in DMD subjects with cardiomyopathy. Future studies correlating cardiomyopathy with ST2 levels may allow for improved non-invasive monitoring of cardiac disease in DMD subjects.",
keywords = "Biomarkers, Cardiomyopathy, Duchenne muscular dystrophy, ST2",
author = "{On Behalf Of The Cinrg Investigators} and Julia Anderson and Haeri Seol and Heather Gordish-Dressman and Yetrib Hathout and Spurney, {Christopher F.} and C. McDonald and McDonald, {Craig M} and E. Henricson and Henricson, {Erik K} and E. Goude and L. Johnson and J. Han and N. Joyce and Nanette Joyce and V. Viswanathan and C. Chidambaranathan and S. Kumar and V. Lakshmi and P. Reddappa and D. Biggar and L. McAdam and M. Dermody and L. Eliasoph and V. Harris. and G. Lee and J. Mah and A. Chiu and T. Haig and M. Harris and K. Sanchez and J. Thannhauser and L. Walker and C. Wright and M. Tulinius and A. Alhander and A. Ekstrom and A. Gustafsson and A. Kroksmark and U. Sterky and L. Wahlgren and M. Thangarajh and M. Birkmeier and S. Kaminski and B. Tadesse and A. Toles and A. Kornberg and M. Ryan and K. Carroll and K. DeValle and R. Kennedy",
year = "2017",
month = "8",
day = "18",
doi = "10.1007/s00246-017-1703-9",
language = "English (US)",
pages = "1--7",
journal = "Pediatric Cardiology",
issn = "0172-0643",
publisher = "Springer New York",

}

TY - JOUR

T1 - Interleukin 1 Receptor-Like 1 Protein (ST2) is a Potential Biomarker for Cardiomyopathy in Duchenne Muscular Dystrophy

AU - On Behalf Of The Cinrg Investigators

AU - Anderson, Julia

AU - Seol, Haeri

AU - Gordish-Dressman, Heather

AU - Hathout, Yetrib

AU - Spurney, Christopher F.

AU - McDonald, C.

AU - McDonald, Craig M

AU - Henricson, E.

AU - Henricson, Erik K

AU - Goude, E.

AU - Johnson, L.

AU - Han, J.

AU - Joyce, N.

AU - Joyce, Nanette

AU - Viswanathan, V.

AU - Chidambaranathan, C.

AU - Kumar, S.

AU - Lakshmi, V.

AU - Reddappa, P.

AU - Biggar, D.

AU - McAdam, L.

AU - Dermody, M.

AU - Eliasoph, L.

AU - Harris., V.

AU - Lee, G.

AU - Mah, J.

AU - Chiu, A.

AU - Haig, T.

AU - Harris, M.

AU - Sanchez, K.

AU - Thannhauser, J.

AU - Walker, L.

AU - Wright, C.

AU - Tulinius, M.

AU - Alhander, A.

AU - Ekstrom, A.

AU - Gustafsson, A.

AU - Kroksmark, A.

AU - Sterky, U.

AU - Wahlgren, L.

AU - Thangarajh, M.

AU - Birkmeier, M.

AU - Kaminski, S.

AU - Tadesse, B.

AU - Toles, A.

AU - Kornberg, A.

AU - Ryan, M.

AU - Carroll, K.

AU - DeValle, K.

AU - Kennedy, R.

PY - 2017/8/18

Y1 - 2017/8/18

N2 - Duchenne muscular dystrophy (DMD) is a rare, fatal X-linked disorder characterized by the lack of dystrophin, a key sarcolemma muscle protein. Cardiac failure is a significant cause of death in DMD subjects. The purpose of our research was to identify potential cardiac serum biomarkers associated with DMD cardiomyopathy. This is an observational, case-controlled study using subjects from the CINRG DMD natural history study with cardiomyopathy (ejection fraction (EF) <55%; shortening fraction (SF) <28%), subjects without cardiomyopathy (EF ≥ 55%; SF ≥ 28%) compared to normal healthy volunteer subjects. The DMD with cardiomyopathy group had significantly lower average EF and SF (EF = 45 ± 10/SF = 25 ± 2%) than the DMD without cardiomyopathy group (EF = 58 ± 5% and SF = 32 ± 3%; p < 0.01). Among a selected set of potential biomarkers for cardiomyopathy (MMP9, BNP, GAL3, CRP, LEP, TNC, TLR4 and ST2) we validated ST2 as significantly elevated in the serum of DMD cardiomyopathy group (35,798 ± 4884 pg/mL) compared to normal controls (9940 ± 2680 pg/mL; p < 0.01; n = 6). Matrix metallopeptidase 9 (MMP9) levels were found significantly increased in both DMD groups compared to controls (p < 0.01). No significant differences were seen in BNP, GAL3, CRP, LEP, TNC or TLR4 levels. Increased ST2 levels were found in serum of DMD subjects compared to healthy volunteers and further elevated in DMD subjects with cardiomyopathy. Future studies correlating cardiomyopathy with ST2 levels may allow for improved non-invasive monitoring of cardiac disease in DMD subjects.

AB - Duchenne muscular dystrophy (DMD) is a rare, fatal X-linked disorder characterized by the lack of dystrophin, a key sarcolemma muscle protein. Cardiac failure is a significant cause of death in DMD subjects. The purpose of our research was to identify potential cardiac serum biomarkers associated with DMD cardiomyopathy. This is an observational, case-controlled study using subjects from the CINRG DMD natural history study with cardiomyopathy (ejection fraction (EF) <55%; shortening fraction (SF) <28%), subjects without cardiomyopathy (EF ≥ 55%; SF ≥ 28%) compared to normal healthy volunteer subjects. The DMD with cardiomyopathy group had significantly lower average EF and SF (EF = 45 ± 10/SF = 25 ± 2%) than the DMD without cardiomyopathy group (EF = 58 ± 5% and SF = 32 ± 3%; p < 0.01). Among a selected set of potential biomarkers for cardiomyopathy (MMP9, BNP, GAL3, CRP, LEP, TNC, TLR4 and ST2) we validated ST2 as significantly elevated in the serum of DMD cardiomyopathy group (35,798 ± 4884 pg/mL) compared to normal controls (9940 ± 2680 pg/mL; p < 0.01; n = 6). Matrix metallopeptidase 9 (MMP9) levels were found significantly increased in both DMD groups compared to controls (p < 0.01). No significant differences were seen in BNP, GAL3, CRP, LEP, TNC or TLR4 levels. Increased ST2 levels were found in serum of DMD subjects compared to healthy volunteers and further elevated in DMD subjects with cardiomyopathy. Future studies correlating cardiomyopathy with ST2 levels may allow for improved non-invasive monitoring of cardiac disease in DMD subjects.

KW - Biomarkers

KW - Cardiomyopathy

KW - Duchenne muscular dystrophy

KW - ST2

UR - http://www.scopus.com/inward/record.url?scp=85027722974&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85027722974&partnerID=8YFLogxK

U2 - 10.1007/s00246-017-1703-9

DO - 10.1007/s00246-017-1703-9

M3 - Article

C2 - 28821969

AN - SCOPUS:85027722974

SP - 1

EP - 7

JO - Pediatric Cardiology

JF - Pediatric Cardiology

SN - 0172-0643

ER -