CSB327H1 Lecture Notes - Lecture 6: Tenser, Primary And Secondary Antibodies, Hemidesmosome
Document Summary
A3(iv)a4(iv)a5(iv) has more crosslinking that increases tensile strength. Forms a tenser network than embryonic gbm a1(iv)2a2(iv) More common in males (x-linked) mutation in col4a5. Mutations in col4a3 and col4a4 are located on autosomes. Thickening and falling apart of gbm (blocking of filtration slits) Kidney failure (blood in urine), high blood pressure. Take serum from patient, make a primary antibody from it. A6b4 (in basal, beta chain sticks into hemidesmosome) binds to laminin. 5 laminin binds to collagen iv (dermis) collagen vii loops around collagen i & iii and back into the lamina densa. Antiparallel dimer at c-terminal c-terminal cleavage (disulfide bonds) Binding of n domain to type i and type iv collagen. Autoantibody against type vii collagen (globular n terminal) Mucous membranes (orifices,eyes,gastrointestinal and respiratory membrane, internal tissue, placenta) Transmembrane collagen: ectodomain there are repeats of rod-like collagenous domains. C-termini is a coiled-coiled structure in the basement membrane. Binds to a6b4 integrin (associated with collagen vii) and laminin 5.
