Nerlich A G, Brenner R E, Wiest I, Lehmann H, Yang C, Müller P K, von der Mark K
Department of Pathology, University of Munich, Germany.
Am J Med Genet. 1993 Jan 15;45(2):258-9. doi: 10.1002/ajmg.1320450221.
Immunohistochemical studies of bone from individuals with osteogenesis imperfecta (OI) type II, OI type III, or OI type IV demonstrate a similar pattern, but varying extent, of the abnormal presence of interstitial collagens in bone matrix. OI type II bone had nests of cartilage with type II collagen, and significant type III collagen in the bone matrix. In OI types III and IV, type II collagen was present only in epiphyseal cartilage but bone still contained type III collagen. These findings resembled those in developing fetal bone indicating the "immature" nature of OI bone.
对Ⅱ型、Ⅲ型或Ⅳ型成骨不全症(OI)患者的骨骼进行免疫组织化学研究表明,骨基质中间质胶原异常存在的模式相似,但程度不同。Ⅱ型OI骨有含Ⅱ型胶原的软骨巢,且骨基质中有大量Ⅲ型胶原。在Ⅲ型和Ⅳ型OI中,Ⅱ型胶原仅存在于骨骺软骨中,但骨中仍含有Ⅲ型胶原。这些发现与发育中的胎儿骨骼相似,表明OI骨具有“不成熟”的性质。