Molecular consequences of the SERPINH1/HSP47 mutation in the dachshund natural model of osteogenesis imperfecta

Journal of Biological Chemistry

Volumn 290, Issue 29, 2015, Pages 17679-17689

  Lindert, Uschi ^a   Weis, Mary Ann ^b   Rai, Jyoti ^b   Seeliger, Frank ^c   Hausser, Ingrid ^d   Leeb, Tosso ^e   Eyre, David ^b   Rohrbach, Marianne ^a   Giunta, Cecilia ^a  

^a UNIVERSITY CHILDREN S HOSPITAL   (Switzerland)

^b UNIVERSITY OF WASHINGTON SCHOOL OF MEDICINE   (United States)

^c ASTRAZENECA R AND D   (Sweden)

^d UNIVERSITY HOSPITAL HEIDELBERG   (Germany)

^e UNIVERSITY OF BERN   (Switzerland)

Author keywords

[No Author keywords available]

Indexed keywords

DIAGNOSIS;

BONE COLLAGEN; CHAPERONE FUNCTIONS; ER STRESS; NATURAL MODELS; OSTEOGENESIS IMPERFECTA; POST-TRANSLATIONAL MODIFICATIONS;

COLLAGEN;

COLLAGEN TYPE 1; GLUCOSE REGULATED PROTEIN 78; HEAT SHOCK PROTEIN 47; INITIATION FACTOR 2ALPHA; PROCOLLAGEN; PYRIDINOLINE;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BIOCHEMISTRY; CELL CULTURE; COLLAGEN SYNTHESIS; CONTROLLED STUDY; DACHSHUND (DOG); ELECTROPHORETIC MOBILITY; ENDOPLASMIC RETICULUM; ENDOPLASMIC RETICULUM STRESS; GEL MOBILITY SHIFT ASSAY; GENE MUTATION; HYDROXYLATION; IMMUNOFLUORESCENCE TEST; NONHUMAN; OSTEOGENESIS IMPERFECTA; OVERHYDROXYLATION; POLYACRYLAMIDE GEL ELECTROPHORESIS; PRIORITY JOURNAL; PROTEIN CONTENT; PROTEIN CROSS LINKING; PROTEIN FOLDING; PROTEIN FUNCTION; PROTEIN PROCESSING; PROTEIN SECRETION; SKIN FIBROBLAST; TANDEM MASS SPECTROMETRY; WESTERN BLOTTING; ANIMAL; BONE; CHEMISTRY; DISEASE MODEL; DOG; FIBROBLAST; GENETICS; METABOLISM; PATHOLOGY; POINT MUTATION; PROTEIN STABILITY; VETERINARY;

ANIMALS; BONE AND BONES; CELLS, CULTURED; COLLAGEN TYPE I; DISEASE MODELS, ANIMAL; DOGS; ENDOPLASMIC RETICULUM STRESS; FIBROBLASTS; HSP47 HEAT-SHOCK PROTEINS; OSTEOGENESIS IMPERFECTA; POINT MUTATION; PROTEIN PROCESSING, POST-TRANSLATIONAL; PROTEIN STABILITY;

EID: 84937510633     PISSN: 00219258     EISSN: 1083351X     Source Type: Journal    
DOI: 10.1074/jbc.M115.661025     Document Type: Article

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