TY  - JOUR
AU  - Duran, Ivan
AU  - Zieba, Jennifer
AU  - Csukasi, Fabiana
AU  - Martin, Jorge H
AU  - Wachtell, Davis
AU  - Barad, Maya
AU  - Dawson, Brian
AU  - Fafilek, Bohumil
AU  - Jacobsen, Christina M
AU  - Ambrose, Catherine G
AU  - Cohn, Daniel H
AU  - Krejci, Pavel
AU  - Lee, Brendan H
AU  - Krakow, Deborah
PY  - 2022
DO  - 10.1002/jbmr.4501
UR  - http://hdl.handle.net/10668/22150
T2  - Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research
AB  - Osteogenesis imperfecta (OI) is a genetically heterogenous disorder most often due to heterozygosity for mutations in the type I procollagen genes, COL1A1 or COL1A2. The disorder is characterized by bone fragility leading to increased fracture...
LA  - en
KW  - 4-PBA
KW  - Aga2
KW  - Bip+/−
KW  - Chop−/−
KW  - ER stress
KW  - bone
KW  - osteogenesis imperfecta
KW  - Animals
KW  - Butylamines
KW  - Collagen Type I
KW  - Disease Models, Animal
KW  - Mice
KW  - Molecular Chaperones
KW  - Mutation
KW  - Osteoblasts
KW  - Osteogenesis
KW  - Osteogenesis Imperfecta
KW  - Phenotype
TI  - 4-PBA Treatment Improves Bone Phenotypes in the Aga2 Mouse Model of Osteogenesis Imperfecta.
TY  - research article
VL  - 37
ER  -