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EXTRACELLULAR MATRIX - Coggle Diagram

- - - - TYPE I = Noncartilaginous CT (i.e. bone, tendon, skin)
        TYPE II = Cartilage, vitreous humor
        TYPE III = Extensible CT (i.e. skin, lungs, VASCULAR system)
        TYPE IV = Basement membrane
        TYPE VI = Muscles, Most CTs
        TYPE VII = Dermal-epidermal junction
      - Structure: (Gly-X-Y)n,
        wherein X = mostly Pro (can be Lys) and
        Y = mostly hydroxyPro (can be hydroxyLys)
      - Clinical Correlations
        
        Alport Syndrome / Hereditary Nephritis
        Defect: Type IV
        Features:
        
        Hematuria
        
        Ocular lesions
        
        Hearing loss
        
        Kidney failure
        
        Epidermolysis Bullosa
        Defect: Type VII
        Feature:
        
        Skin breaks and blisters easily
        
        Ehler-Danlos Syndrome
        Features:
        
        Hyperextensibility of the skin
        
        Abnormal tissue fragility
        
        Increased joint mobility
        
        TYPES
        
        Hypermobile = Most common ; Joint hypermobility
        
        Classical = Abnormality in Type V
        
        Vascular = Most serious type ; Abnormalities in Type III ; Fragile BVs
        
        Scurvy
        
        Vitamin C deficiency
        
        Osteogenesis Imperfecta
        Defect: Type I
        Feature: Fragile bones that fracture easily
        
        Menkes Disease
        
        Copper deficiency
    - - Clinical
        Correlation
        
        Marfan Syndrome
        
        Mutation in the FBN1 gene >>> Defect in fibrillin-1
        
        Features:
        
        Tall w/ long extremities
        
        Pectus carinatum
        
        Arachnodactyly
        
        Long, tapering fingers and toes
        
        Predisposition to aortic root, aneurysm rupture, or dissection