Table 2.

Biomarkers related to the bone system in MPS.

	Increased	Decreased
Bone remodeling biomarkers	Osteocalcin, BSAP, urinary PYD, and DYD (MPS I, II, VI) [60]
Pro-Inflammatory biomarkers	IL1β, IL6, TNF-α,TGF-β, NO, and EGF (MPSII, IVA) [59] MMP-1,MIP-1 and MMP-9 (MPS IVA) [57]	MMP-2 (MPS IVA) [61]
Transcriptional and genetic factors	SOX9 (MPS VII), COL2A1, NKX3-2 [42]	RUNX2, ALPL, BGLAP, COL10A1, osteopontin (SPP1) (MPSVII) [42]
Expressed protein biomarkers	MMP-2, -9,TIMP-1 (joint arthritis marker) [62] Lys-O-Gal/Lys-O-GalGlc (MPSI, II, IV) (collagen degradation marker) [63]	FOXA2, MMP13, PTCH1, PTH1R (MPS VII) [42] (chondrocyte maturation marker) TypeII A Collagen (MPSII, IVA) [64]
Other new biomarkers in each MPS	Collagen alpha-1 chain, Fatty acid-binding protein 5, Nidogen, cartilage oligomeric matrix protein, IGFBP7 (MPSI,IIneurological form) [61], βgalactosidase (MPSI, II neurological form, VI) [64] Glycosylated hydroxylysines (MPSIVA) A1T1, lipoprotein (a), SAA, clusterin and vitronectin (MPS IVA) [62] Protein HEG homolog1 (MPS VI) [64]	Fetuin-A (IVA) [63]