3edh
Crystal structure of bone morphogenetic protein 1 protease domain in complex with partially bound DMSOCrystal structure of bone morphogenetic protein 1 protease domain in complex with partially bound DMSO
Structural highlights
Disease[BMP1_HUMAN] Defects in BMP1 are the cause of osteogenesis imperfecta 13 (OI13) [MIM:614856]. An autosomal recessive form of osteogenesis imperfecta, a connective tissue disorder characterized by bone fragility, low bone mass, and recurrent fractures. OI13 is characterized by normal teeth, faint blue sclerae, severe growth deficiency, borderline osteoporosis, severe bone deformity, and recurrent fractures affecting both upper and lower limbs.[1] [2] Function[BMP1_HUMAN] Cleaves the C-terminal propeptides of procollagen I, II and III. Induces cartilage and bone formation. May participate in dorsoventral patterning during early development by cleaving chordin (CHRD). Responsible for the proteolytic activation of lysyl oxidase LOX. Evolutionary Conservation![]() Check, as determined by ConSurfDB. You may read the explanation of the method and the full data available from ConSurf. See AlsoReferences
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Proteopedia Page Contributors and Editors (what is this?)Proteopedia Page Contributors and Editors (what is this?)
OCA- Human
- Procollagen C-endopeptidase
- Sweeney, A Mac
- Alternative splicing
- Calcium
- Chondrogenesis
- Cleavage on pair of basic residue
- Cytokine
- Developmental protein
- Differentiation
- Egf-like domain
- Glycoprotein
- Growth factor
- Hydrolase
- Metal-binding
- Metalloprotease
- Osteogenesis
- Polymorphism
- Protease
- Vicinal disulfide
- Zinc
- Zymogen