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Bone Res. 2020 Mar 9;8:13. doi: 10.1038/s41413-020-0089-0. eCollection 2020.

The cartilage matrisome in adolescent idiopathic scoliosis.

Author information

1
1Center for Pediatric Bone Biology and Translational Research, Texas Scottish Rite Hospital for Children, 2222 Welborn St., Dallas, TX 75219 USA.
2
2McDermott Center for Human Growth and Development, University of Texas Southwestern Medical Center, Dallas, TX 75235 USA.
3
3Departments of Pediatrics, University of Texas Southwestern Medical Center, Dallas, TX 75235 USA.
4
4Orthopaedic Surgery, University of Texas Southwestern Medical Center, Dallas, TX 75235 USA.
5
5Department of Developmental Biology, Washington University School of Medicine, St. Louis, MO 63110 USA.
6
6Department of Bioengineering and Therapeutic Sciences, University of California San Francisco, San Francisco, CA 94158 USA.
7
7Institute for Human Genetics, University of California San Francisco, San Francisco, CA 94158 USA.
8
8Department of Anatomy and Cell Biology, University of Florida, College of Medicine, Gainesville, FL 32610 USA.
9
9Departments of Neurology, Washington University School of Medicine, St Louis, MO 63110 USA.
10
10Pediatrics, Washington University School of Medicine, St Louis, MO 63110 USA.
11
11Orthopaedic Surgery, Washington University School of Medicine, St Louis, MO 63110 USA.
12
12Department of Pediatrics, Dell Pediatric Research Institute, University of Texas at Austin Dell Medical School, Austin, TX 78723 USA.

Abstract

The human spinal column is a dynamic, segmented, bony, and cartilaginous structure that protects the neurologic system and simultaneously provides balance and flexibility. Children with developmental disorders that affect the patterning or shape of the spine can be at risk of neurologic and other physiologic dysfunctions. The most common developmental disorder of the spine is scoliosis, a lateral deformity in the shape of the spinal column. Scoliosis may be part of the clinical spectrum that is observed in many developmental disorders, but typically presents as an isolated symptom in otherwise healthy adolescent children. Adolescent idiopathic scoliosis (AIS) has defied understanding in part due to its genetic complexity. Breakthroughs have come from recent genome-wide association studies (GWAS) and next generation sequencing (NGS) of human AIS cohorts, as well as investigations of animal models. These studies have identified genetic associations with determinants of cartilage biogenesis and development of the intervertebral disc (IVD). Current evidence suggests that a fraction of AIS cases may arise from variation in factors involved in the structural integrity and homeostasis of the cartilaginous extracellular matrix (ECM). Here, we review the development of the spine and spinal cartilages, the composition of the cartilage ECM, the so-called "matrisome" and its functions, and the players involved in the genetic architecture of AIS. We also propose a molecular model by which the cartilage matrisome of the IVD contributes to AIS susceptibility.

KEYWORDS:

Bone quality and biomechanics; Pathogenesis

Conflict of interest statement

Competing interestsThe authors declare no competing interests.

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