Collagen Types and Linked Disorders

There are 29 genetically distinct collagens present in animal tissues. Collagen types I, II, III, V and XI self-assemble into D-periodic cross-striated fibrils. Here the D is approximately 67 nm and there is characteristic axial periodicity of collagen. These form the most abundant collagens in vertebrates.

Collagen types I – V

  • Type I collagen is found throughout the body except in cartilaginous tissues. It is found in skin, tendon, vascular, ligature, organs and is the main component of bone. It is also synthesized in response to injury and in the fibrous nodules in fibrous diseases. Over 90% of the collagen in the body is type I.
  • Type II collagen is the main component of cartilage. It is also found in developing cornea and vitreous humour. These are formed from two or more collagens or co-polymers rather than a single type of collagen.
  • Type III collagen is found in the walls of arteries and other hollow organs and usually occurs in the same fibril with type I collagen.
  • Type IV forms the bases of cell basement membrane
  • Type V collagen and type XI collagen are minor components of tissue and occur as fibrils with type I and type II collagen respectively. Type V forms cell surfaces, hair and placenta.

Collagen linked diseases

Due to the diverse location of the collagen types they are associated with diseases. The collagen linked diseases commonly arise from genetic defects or nutritional deficiencies. These defects often cause problems in the biosynthesis of the collagen molecules, their assembly and posttranslational modification process that makes them their final end form of collagen.

There may be defects in collagen secretion and general production as well. In addition there is excess collagen formation and deposition in scleroderma.

Collagen-related diseases most commonly arise from genetic defects or nutritional deficiencies that affect the biosynthesis, assembly, postranslational modification, secretion, or other processes involved in normal collagen production.

TypeNotesGene(s)Disorders
IThis is the most abundant collagen of the human body. It is present in scar tissue, the end product when tissue heals by repair. It is found in tendons, skin, artery walls, the endomysium of myofibrils, fibrocartilage, and the organic part of bones and teeth.COL1A1, COL1A2osteogenesis imperfecta, Ehlers-Danlos Syndrome, Infantile cortical hyperostosis aka Caffey's disease
IIHyaline cartilage, makes up 50% of all cartilage protein. Vitreous humour of the eye.COL2A1Collagenopathy
IIIThis is the collagen of granulation tissue, and is produced quickly by young fibroblasts before the tougher type I collagen is synthesized. Reticular fiber. Also found in artery walls, skin, intestines and the uterusCOL3A1Ehlers-Danlos Syndrome
IVbasal lamina; eye lens. Also serves as part of the filtration system in capillaries and the glomeruli of nephron in the kidney.COL4A1, COL4A2, COL4A3, COL4A4, COL4A5, COL4A6Alport syndrome, Goodpasture's syndrome
Vmost interstitial tissue, assoc. with type I, associated with placentaCOL5A1, COL5A2, COL5A3Ehlers-Danlos syndrome (Classical)
VImost interstitial tissue, assoc. with type ICOL6A1, COL6A2, COL6A3Ulrich myopathy and Bethlem myopathy
VIIforms anchoring fibrils in dermal epidermal junctionsCOL7A1epidermolysis bullosa dystrophica
VIIIsome endothelial cellsCOL8A1, COL8A2Posterior polymorphous corneal dystrophy 2
IXFACIT collagen, cartilage, assoc. with type II and XI fibrilsCOL9A1, COL9A2, COL9A3– EDM2 and EDM3
Xhypertrophic and mineralizing cartilageCOL10A1Schmid metaphyseal dysplasia
XIcartilageCOL11A1, COL11A2Collagenopathy
XIIFACIT collagen, interacts with type I containing fibrils, decorin and glycosaminoglycansCOL12A1
XIIItransmembrane collagen, interacts with integrin a1b1, fibronectin and components of basement membranes like nidogen and perlecan.COL13A1
XIVFACIT collagenCOL14A1
XVCOL15A1
XVICOL16A1
XVIItransmembrane collagen, also known as BP180, a 180 kDa proteinCOL17A1Bullous pemphigoid and certain forms of junctional epidermolysis bullosa
XVIIIsource of endostatinCOL18A1
XIXFACIT collagenCOL19A1
XXCOL20A1
XXIFACIT collagenCOL21A1
XXIICOL22A1
XXIIIMACIT collagen –COL23A1
XXIVCOL24A1
XXVCOL25A1
XXVIEMID2
XXVIICOL27A1
XXVIIICOL28A1
XXIXepidermal collagenCOL29A1Atopic dermatitis

In addition to the above mentioned disorders, excessive deposition of collagen occurs in scleroderma.

Sources

  1. http://www.biochem.wisc.edu/faculty/raines/lab/pdfs/Shoulders2009a.pdf
  2. courses.washington.edu/…/Lit_MechPropsCollagen_Fratzl_1997.pdf
  3. http://onlinelibrary.wiley.com/doi/10.1002/bip.360210507/abstract
  4. http://www.biochemj.org/bj/316/0001/3160001.pdf
  5. ueaeprints.uea.ac.uk/…/…ructure_function_and______biosynthesis.pdf
  6. http://www.karinherzog.co.uk/PDF/Articles/Collagen.pdf
  7. http://www.reshapebook.com/ProteinReport.pdf
  8. http://www-personal.umich.edu/~egatenby/collagen%20eyre%20chapter.pdf
  9. http://fabad.org/fabad.org/pdf/volum32/issue3/139-144.pdf

Further Reading

  • All Collagen Content
  • Collagen -What is Collagen?
  • Collagen Molecular Structure
  • Collagen Fibrillar Structure
  • Collagen Synthesis
More…

Last Updated: Apr 19, 2019

Written by

Dr. Ananya Mandal

Dr. Ananya Mandal is a doctor by profession, lecturer by vocation and a medical writer by passion. She specialized in Clinical Pharmacology after her bachelor's (MBBS). For her, health communication is not just writing complicated reviews for professionals but making medical knowledge understandable and available to the general public as well.

Source: Read Full Article