Presentation of fibrillin

  • -is an extracellular protein
  • -is responsible as scafolding for deposition elastic fibres
  • -exists as fibrillin f1 and 2
  • -encoded by FBN1 and 2
  • -encoding Marfan syndrome

 

 

Functions of fibrillin:

  • -strengthens elastic fibres and enables transmission of forces between the epidermis and dermis
  • -What is elastic tissue = elastin + microfibrills fibers

 

 

Elastic tissue differences between age groups 18-30 and over 70

Methodology:

  • -with atomic force microscopy to determine distance between microfibrills (of fibrillin) (periodicty 56nm=no strain, more than 100=strained and unable to strap back= loss of elasticity)
  • -With molecular combing, tensile strength to determine distance and elastic potential

 

 

Results:

  • -Up to 30 years old 0.9% of fibrillin microfibrills are separated by more than 100nm
  • -Over 70 years old this goes up to 18.2 %
  • -on immunohistochemistry buttocks vs photoexposed skin in 7o year old individuals show loss of candelabra structure of fibrillin and also a reduction of fibrillin staining in the papillary dermis

 

 

Mechanisms of age-related damage:

  • -upgrading MMPs (matrix metalloproteinase)
  • -cleavage of proMMP
  • -upregulation MMP 2 and 9
  • -degradation of microfibrills by UV

 

 

By using atomic force and electron microscopy microfibrills of fibrillin respond to  UVB by:

  • -increasing diameter
  • -becoming more tangled (increased flexion angle)
  • -reducing their mass with increasing UV dosage (mass mapping)

 

 

UVR exposed fibrillin fibres are therefore:

  • -shorter
  • -weaker
  • -have a reduced mass

 

 

Treatment

  • -Over 4 years of nightly application, retinoic acid 0,1% reduces photoaging by reconstituting the fibrillin quantity and candelabra disposition.

 

 

Griffiths C, Aging and Photoaging – 22nd World Congress of Dermatology (WCD) – Seoul, South Korea