title: Is low molecular weight hyaluronan an early indicator of disease in avian systemic sclerosis?
abstract: To further elucidate the pathogenesis of systemic sclerosis (SSc) an experimental avian model was used. The University of California at Davis line 200 (UCD-200) chickens spontaneously develop a SSc-like disease that has most features of human SSc with vascular effects, inflammation, autoimmunity, and fibrosis. The first signs of disease in UCD-200 chickens are swelling and ischemic lesions of the comb and the presence of a tissue containing high amounts of glycosaminoglycan hyaluronan (HA). The aim of this study was to evaluate inflammatory and fibrotic processes of the disease with regard to the molecular weight of HA.
  
  
  
  Comb biopsies from UCD-200 and healthy White Leghorn (WL) chickens, as controls, at different ages were studied with the histochemical localization of HA, hyaluronidase-1 (Hyal-1), cluster of differentiation 3, immunoglobulin Y, and collagen I and III. The molecular weight distribution of HA was estimated with gas-phase electrophoretic analysis.
  
  
  
  At 2 days of age, HA was visualized in UCD-200 chickens at the dermal part of the comb with no simultaneous staining of Hyal-1. In adult UCD-200 chickens, the comb skin was almost totally devoid of HA compared to WL chickens of the same age. An increase of low molecular weight (LMW) HA was detected in comb tissue from UCD-200 at the age of 1 day, 1 week, 2 weeks, and 4 weeks, in contrast to adult animals.
  
  
  
  An early inflammatory process involving LMW HA was confirmed as a possible profibrotic process. This indicates that HA might be an important participant in the early inflammatory events of SSc in UCD-200 chickens and that the disappearance of HA in skin predisposes to fibrosis.
authors.affiliation: a Department of Medical Biochemistry and Microbiology , Science for Life Laboratory Uppsala, Uppsala University , Uppsala , Sweden.
authors.forename: Susanne
authors.initials: S
authors.lastname: Kerje
authors.affiliation: b Department of Public Health and Clinical Medicine , Umeå University , Umeå , Sweden.
authors.forename: Urban
authors.initials: U
authors.lastname: Hellman
authors.affiliation: c Department of Medical Chemistry and Biophysics , Unit of Research, Education and Development - Östersund, Umeå University , Umeå , Sweden.
authors.forename: Lan
authors.initials: L
authors.lastname: Do
authors.affiliation: c Department of Medical Chemistry and Biophysics , Unit of Research, Education and Development - Östersund, Umeå University , Umeå , Sweden.
authors.forename: Göran
authors.initials: G
authors.lastname: Larsson
authors.affiliation: d Department of Medicine (Solna) , Karolinska Institutet , Stockholm , Sweden.|e Science for Life Laboratory, Department of Medical Sciences , Uppsala University , Uppsala , Sweden.
authors.forename: Olle
authors.initials: O
authors.lastname: Kämpe
authors.affiliation: b Department of Public Health and Clinical Medicine , Umeå University , Umeå , Sweden.
authors.forename: Anna
authors.initials: A
authors.lastname: Engström-Laurent
authors.affiliation: f Department of Medical Sciences , Uppsala University , Uppsala , Sweden.
authors.forename: Ulla
authors.initials: U
authors.lastname: Lindqvist
mesh_terms: D000825:Animal Structures / Q000378:metabolism / Q000473:pathology; D000818:Animals; D017252:CD3 Complex / Q000378:metabolism; D002645:Chickens; D003094:Collagen / Q000378:metabolism; D018960:Hyaluronan Receptors; D006820:Hyaluronic Acid / Q000737:chemistry* / Q000378:metabolism*; D006821:Hyaluronoglucosaminidase / Q000378:metabolism; D007136:Immunoglobulins / Q000378:metabolism; D007150:Immunohistochemistry; D007249:Inflammation / Q000473:pathology; D008970:Molecular Weight; D012595:Scleroderma, Systemic / Q000175:diagnosis* / Q000473:pathology; D013194:Staining and Labeling; D014867:Water / Q000032:analysis
chemical_list: D017252:CD3 Complex; D018960:Hyaluronan Receptors; C016512:IgY; D007136:Immunoglobulins; D014867:Water; D006820:Hyaluronic Acid; D003094:Collagen; D006821:Hyaluronoglucosaminidase
keywords: Avian model; UCD-200 chicken; hyaluronan; inflammation; systemic sclerosis