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epidermolysis bullosa
N y s t r o m , A . , B o r n e r t , O . , & To b i a s , K . ( 2 0 1 8 ) . I m p a i r e d l y m p h o i d e x t r a c e l l u l a r m a t r i x i m p e d e s . P r o c e e d i n g s o f t h e
National Academy of Sciences of the United States of America, 115 (4), 705-714.
Western Blotting
The underlying cause of the The protein of interest
highly increased bacterial Protein lysates were prepared For immunoprecipitation was placed in gel
colonization of skin and nasal by pulverizing snap-frozen analysis, mouse anti- electrophoresis. Where
cavities suffering from RDEB spleens by using mortar and cochlin is added to the the positive electrode is
was investigated. For the study, pestle, followed by solubilized protein for positioned at the top of
samples from mouse with resuspension in Nonidet P-40– binding to protein of the chamber. While the
molecularly confirmed RDEB containing lysis buffer interest negative electrode is at
were used the bottom.
Cochlin—an ECM protein with expression restricted to
cochlea, lymph nodes, and spleen is of high interest.
Western blot Mutations in the cochlin gene, COCH, result in the
analysis of wild-type deafness, autosomal dominant 9 (DFNA9) syndrome,
and RDEB mouse
spleen lysates for which is a syndrome with adult onset that manifests in
collagen VII and β- progressive hearing loss and vestibular dysfunction The
actin as loading
control
physiological function of cochlin still needs elucidation, but
cochlin has been suggested to be involved in
mechanosensing, as well as being a local and systemic
regulator of antibacterial immunity. Cochlin-deficient mice
show impaired abilities to clear experimentally induced
lung infection with Pseudomonas aeruginosa and
Staphylococcus aureus