Hydroxyhomocitrulline Is a Collagen-Specific Carbamylation Mark that Affects Cross-link Formation

Cell Chem Biol. 2017 Oct 19;24(10):1276-1284.e3. doi: 10.1016/j.chembiol.2017.08.010. Epub 2017 Sep 21.

Abstract

Carbamylation is a non-enzymatic post-translational modification that physiologically occurs during aging and is a risk factor for various diseases. The most common product of carbamylation is homocitrulline (HCit), where a lysine (Lys) amino group has reacted with urea-derived cyanate. HCit has recently been detected in collagen; however, given that 15%-90% of total Lys in collagen is hydroxylated, it is unclear how hydroxylation affects collagen carbamylation. Here, we identified a collagen-specific carbamylation product, hydroxyhomocitrulline (HHCit), and showed that high levels of HHCit are correlated with age in rat tissue collagen and in vivo carbamylation in mice, as well as with the decline of kidney function in the serum of dialysis patients. Proteomic analysis of the carbamylated collagens identified α2(I) Lys933, a major cross-linking site, as a preferential HHCit site. Furthermore, our results suggest that hydroxylysine carbamylation affects the mechanical properties of connective tissue by competitively inhibiting collagen cross-link formation.

Keywords: aging; carbamylation; collagen; cross-link; hydroxylysine; kidney disease.

MeSH terms

  • Aging / metabolism
  • Animals
  • Biomechanical Phenomena
  • Citrulline / analogs & derivatives*
  • Citrulline / metabolism
  • Collagen / chemistry*
  • Collagen / metabolism*
  • Humans
  • Mice
  • Organ Specificity
  • Protein Processing, Post-Translational*
  • Renal Dialysis
  • Urea / metabolism*

Substances

  • homocitrulline
  • Citrulline
  • Urea
  • Collagen