. Author manuscript; available in PMC: 2018 Dec 1.

Published in final edited form as: Ann N Y Acad Sci. 2017 Dec;1410(1):93–106. doi: 10.1111/nyas.13572

Table 2.

Bone Quality Studies in Mouse Models of Osteogenesis Imperfecta

OI Model	Defect	Techniques Used	Characteristic
Type I	Autosomal dominant	Mechanical tests^89,91 pCT⁸⁹ Raman⁸⁹ SAXS⁹² Microhardness⁹² qBEI⁹²	Reduced mechanical properties⁹¹ Sex-dependent composition⁸⁹ Reduced hydroxyproline content⁸⁹ Increased hardness⁹²
+/oim	Autosomal dominant
Type III	Autosomal dominant; Qualitative-splicing mutation; glycine substitutions	XRD⁷⁷ FTIRI^{78,79,80,81,90} SHG⁸⁸ Raman^84,89 Mechanical tests⁹⁰	Crystals smaller^77,80 Hypermineralized^78,79,80,81 Decreased CO3/PO4^78,79,80 Increased collagen x-links^78,79,80 Crystals not well aligned with collagen⁸⁴; sex-dependent composition⁸⁹; reduced hydroxyproline content⁸⁹; reduced material properties⁹⁰ Poorly aligned collagen ⁸¹ Decreased Ca content Brittle⁹⁰
oim/oim
G610C (Amish)
Type IV	Autosomal recessive; Qualitative	Raman⁸⁶ microCT⁸⁶ AFM⁸⁷	Reduced cortical thickness⁸⁶ Less fracture resistant up to 6 mo. of age; mineral/matrix reduced at 6 mo⁸⁶; Collagen d- spacing altered⁸⁷
Brtl	Autosomal recessive; Qualitative	Raman⁸⁶ microCT⁸⁶ AFM⁸⁷
Type VI	Autosomal recessive	Micro-CT, FTIRI, histomorphometry⁹³ SAXS and BMDD⁸²	Reduced trabecular bone volume; accumulation of unmineralized bone; increased mineral/matrix ⁹³ increased Ca content, altered particle alignment with collagen⁸²
PEDF^−/−		Micro-CT, FTIRI, histomorphometry⁹³ SAXS and BMDD⁸²
Fro/fro		FTIRI⁷⁸ uCT⁷⁸	Decreased Min/Mat⁷⁸; decreased cross-linking⁷⁸
Type VII Crtap^−/−	Autosomal recessive Defective 3′-OH- prolylase complex	Micro-CT^85,81 Raman ⁸⁵ Mechanical tests ⁸⁵ FTIRI⁸¹ Backscattered electron imaging ⁸³	Hypermineralization; increased mineral/matrix; decreased crystal size; sex-dependent differences in material properties⁸¹