Bone 140 (2020) 115548 Contents lists available at ScienceDirect Bone journal homepage: www.elsevier.com/locate/bone Review Article Discovery of bone morphogenetic proteins – A historical perspective a,⁎ T b T. Kuber Sampath , A. Hari Reddi a perForm biologics Inc., Holliston, MA, United States of America Lawrence Ellison Center for Musculoskeletal Regeneration, Department of Orthopedic Surgery, School of Medicine, University of California at Davis, Sacramento, CA, United States of America b A R T I C LE I N FO A B S T R A C T Keywords: BMP Bone repair Review Bone morphogenetic proteins (BMPs) were purified from demineralized bone matrix by their ability to induce new bone formation in vivo. BMPs represent a large sub-family of proteins structurally related to TGF-beta and activins. Two BMP bone graft substitutes, BMP2 (InFuse®) and BMP7 (OP1®) have been developed as products for the repair of long bone non-union fractures and lumbar spinal fusion in humans. The approval of BMP2 and BMP7 based products for use in the clinic supports that the signals responsible for bone formation at ectopic sites can form a basis as therapeutics for bone repair and regeneration. This article describes a historical perspective of the discovery BMPs. 1. Early history Bone has natural capacity for healing upon fractures. The use of antiseptic decalcified bone has long been realized [1,2] for the construction of bone following osteomyelitis and applied it to repair bone deformities. Huggins [3,4] demonstrated for the first time an experimental means of inducing new bone at ectopic non-bony sites and showed that urinary bladder epithelium when implanted under abdominal fascia in dogs resulted in osteoblast differentiation locally as evidenced by the presence of high alkaline phosphatase activity and accumulation of hydroxyapatite containing mineralized nodules. He described this phenomenon as “Epithelial Osteogenesis”. This finding showed that “live” epithelium provided stimulus (signal) for the adjacent connective tissue fibroblast-like cells (cells) to respond under a permissive tissue-matrix environment (scaffold) to form bone. It has now been realized that three biological principles required for tissue engineering are: signals, cells and scaffold. 2. Bone induction by demineralized bone matrix Urist discovered that demineralized bone is capable of inducing new bone when implanted ectopically in the skeletal muscle [5] and he described this phenomenon as “Bone Formation by Autoinduction”. His observation was the first proof that acellular demineralized bone matrix has a bone morphogenic activity as it is capable of inducing new bone at ectopic sites. Urist and Huggins showed that demineralized dentin matrix is also capable of inducing new bone in vivo [6]. Urist and Strates ⁎ later named the bone- and dentin- derived substances as “Bone Morphogenetic Proteins” [7]. Independently, it was shown that allogenic demineralized bone matrix when implanted in a rat subcutaneous site initiates a cascade of cellular events mimicking the biological processes of embryonic bone formation [8,9]. This matrix-induced new bone formation involves migration, proliferation of fibroblast-like mesenchymal stem cells and their differentiation into chondrocytes to form cartilage as a transient tissue which upon calcification and concurrently with vascular ingrowth formed new bone and bone marrow at predictable time intervals in a reproducible manner. As a result, it was believed that bone matrix likely contained a set of morphogenic proteins responsible for the cascade of cellular events associated with matrix-induced new bone formation. 3. Discovery of bone morphogenetic proteins The progress in identifying bone morphogenetic protein from the demineralized bone matrix has been slow due to the difficulty in isolating protein from the insoluble bone matrix and not having a defined bioassay for in vivo bone formation. However, it was eventually demonstrated that proteins responsible for bone formation could be solubilized from the demineralized bone matrix by dissociative extraction using 4 M guanidium chloride or 8 M urea containing 1 M NaCl or 1% SDS at neutral pH over 16 h. The extracted proteins could be reconstituted with an insoluble residual inactive collagenous matrix and assayed their bone forming ability in a rat subcutaneous site [10] (Fig. 1). In this dissociative extraction and reconstitution assay, the Corresponding author. E-mail address: kuber.sampath@performbiologics.com (T.K. Sampath). https://doi.org/10.1016/j.bone.2020.115548 Received 24 June 2020; Received in revised form 3 July 2020; Accepted 6 July 2020 Available online 27 July 2020 8756-3282/ © 2020 Elsevier Inc. All rights reserved. Bone 140 (2020) 115548 T.K. Sampath and A.H. Reddi Fig. 1. A copy of the discussion note describing the steps to perform dissociative extraction and reconstitution and the data obtained from the rat subcutaneous implants on Day 12. Sampath TK, Reddi AH. (1981) Proc Natl Acad Sci USA 78:7599–7602. solubilized bone extract provided the signals and the inactive residual collagenous matrix served as scaffold and the subcutaneous ectopic site recruited the responding mesenchymal stem cells to undergo proliferation and differentiation into endochondral bone formation. This advance for the first time provided not only a reproducible bioassay for bone morphogenetic protein but also helped to qualify that the bone forming activity is homologous among the mammals [11]. These findings permitted the isolation, characterization and identification of several bone morphogenetic proteins from bone matrix extract. By utilizing the amino acid sequences obtained from enriched bone inductive protein fractions isolated from bovine bone [12], several genes encoding “putative” bone morphogenetic proteins (BMP1, −2, −3 and −4) were identified by molecular cloning techniques. Of them, BMP2, −3 and −4 were members of the TGF-beta family of proteins. BMP1 was a mammalian tolloid proteinase responsible for processing extracellular matrix proteins like collagens and certain member of the TGF-beta family of proteins including TGF-beta and GDFs, and was later found to be a contaminant. Independently, the bovine osteogenic protein was highly purified and shown to be composed of homodimers of an 18 kDa protein and a 16 kDa protein [13] (Fig. 2). The purified bovine osteogenic protein by SDS-PAGE gel and 125Ilabeled, was divided in half and electrophoresed either untreated (oxidized) or after reduction (reduced & pyridylathylated). Based on amino acid sequences obtained from these highly purified bovine osteogenic proteins, a relationship to Drosophila DPP and Xenopus Vg-1 was established. As a result, a consensus gene construct was assembled and used to obtain the OP-1 gene for the first time [14]. The OP-1 gene was later renamed as BMP7 [15]. Subsequently, the 18 and 16 kDa proteins were identified as homodimers of OP-1 and BMP2, respectively [13]. Later, several additional BMP-related genes were cloned [16] from human cDNA and genomic libraries using oligonucleotide probes whose constructions were based on known BMP gene sequences. These genes were called Growth and Differentiation Factors (GDFs). Independently, other researchers have identified a set of morphogenetic proteins called cartilage-derived morphogenetic proteins, CDMPs (also called CDMP1/GDF-5/BMP11; CDMP-2/GDF-6/BMP12; CDMP-3/GDF-7/BMP13) as they were shown to be expressed predominantly in cartilage [17]. Fig. 2. Purification of highly purified Bovine Osteogenic Protein. 4. BMP structure and function Like for all members of the TGF-beta superfamily, BMPs are synthesized as a large precursor and then processed as a mature disulfide linked dimer. For a BMP to be active dimerization is a requirement [18,19]. The products approved for clinical use employ mature disulfide-linked BMP homodimer applied locally in combination with a collagenous scaffold. BMP exerts its function by binding to a specific Ser/Thr kinase receptor heterodimeric complex composed of one type I receptor and one type II receptor [20–22]. The binding of BMP ligand to 2 Bone 140 (2020) 115548 T.K. Sampath and A.H. Reddi A B C D Fig. 3. Photomicrographs of the OP-1/BMP7 induced endochondral bone formation in the rat subcutaneous implants (stained with toluidine blue). (For interpretation of the references to color in this figure legend, the reader is referred to the web version of this article.) they are expressed in tissues other than bone [31,32]. BMPs serve as inductive signals for a number of tissues during organogenesis, suggesting that they may have therapeutic utility in tissues other than bone. For example, BMP2 has a developmental role in cardiac tissue [33], BMP4 (a structurally close member to BMP2) in lung development [34] and BMP7 in kidney morphogenesis [35,36]. The loss of BMP6 exhibits hemochromatosis [37] and gain of BMP6 function results in anemia through disturbance of the iron-hemojuvelin-hepcidin loop. The loss of GDF-8 results in enhanced skeletal myogenesis with high metabolic activity exhibiting a lean phenotype [38], whereas GDF-11 (closely related to GDF-8) appears to have a positive role in the ageing process [39]. its receptor complex may also be facilitated by extracellular matrix proteins like Type IV collagen and Heparan Sulfate proteoglycan and co receptors that include Endoglin and Hemojuvelin. The ligand-receptor complex subsequently induces the phosphorylation of intracellular effector proteins SMAD1/5/8 to mediate downstream signaling. The binding of BMP to its receptor complex is tightly controlled in the extracellular milieu by endogenous antagonists [23] (e.g., Noggin). Recombinant human BMP when implanted with allogeneic collagenous matrix is capable of inducing new bone at ectopic sites in a dose-dependent manner [24]. BMP2 [25,26], OP-1/BMP7 [27,28] and BMP6 [29,30] all have been shown to induce new bone formation in the rat subcutaneous implants, restore large segmental defects in rabbits when implanted with a collagenous scaffold. The doses employed vary based on the specific BMP and the substratum used as carriers. The efficacy of BMP2, −6 and −7 has been shown to exhibit a comparable bone forming activity in the rat subcutaneous implant assay and this activity is dose-dependent. A single BMP is sufficient to induce the cascade of cellular events leading to the formation of new bone at ectopic sites, an example was shown for OP-1/BMP7 in Fig. 3, as well as it elicits this response in diaphyseal segmental defect models of small and large animals [27,28]. A, negative control (Day12), rat bone collagenous matrix (25 mg) as carrier. B, rat collagenous carrier consituted with 125 ng of rhBMP7 (Day 7), evidence of chondrogeneis is seen. C, rat collageneous carrier with rhBMP7 (Day12), note the extensive bone formation and remodeling. D, rat collagenous carrier plus rhBMP7 (Day21), note hematopoietic bone marrow differentiaitn in the newly formed ossicles. While BMPs are capable of forming bone at ectopic and bony sites, 5. BMP clinical studies Several clinical trials have been conducted to assess the safety and efficacy of recombinant BMP containing devices for the treatment of acute diaphyseal bone fractures, delayed union, tibial nonunion and spinal fusion. Two BMP products, rhBMP2 (InFUSE® [40] and Amplify®) [41] and rhBMP7 (OP-1 Implant® [42] and OP-1 Putty® [43]) are licensed under a PMA and HDE, respectively, for marketing and clinical use in the U.S. BMP, as an injectable drug, is not efficient for local bone formation. It therefore required delivery using an appropriate carrier to induce new bone formation at local implant sites. Collagenous matrix not only serves as a carrier for BMPs but also acts as a substratum to facilitate mesenchymal cell recruitment and proliferation and subsequently differentiation into endochondral bone formation. While it is preferred to 3 Bone 140 (2020) 115548 T.K. Sampath and A.H. Reddi use autologous or allogenic collagenous matrix as carrier/substratum to minimize immune responses in humans, thus far the clinically approved BMP-based osteogenic devices have routinely employed xenogeneic (bovine-sourced) collagenous matrices and/or bovine collagen-mineral composites. The collagen-mineral composite carrier exhibits a weak affinity for rhBMPs and invariably provokes an inflammatory and foreign body giant cell responsiveness at the implant site, due to the high mineral content. In order to overcome these insults, high doses of rhBMPs were employed. For example, rhBMP2 is applied in 12–40 mg doses for single-level posterolateral lumbar fusion [41,44] resulting in local and systemic safety issues and an unwanted clinical outcome. A preferred carrier for BMP would be an autologous physiological carrier which does not provoke an inflammatory and immune responses and exhibits a high affinity for BMPs. Hence low doses of BMPs could be employed to induce bone formation without causing any unwanted safety issues. Recent studies have shown autologous blood coagulum (ABC) can serve as a physiological carrier for BMPs. An autologous bone graft substitute (ABGS) that contains rhBMP6 and ABC was capable of inducing new bone formation in a rat subcutaneous site [29], repairing diaphyseal segmental defect in rabbits [29] and promoting posterolateral lumbar fusion in rabbit [30] and sheep [45] models. 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Vukicevic, Recombinant human bone morphogenetic protein 6 delivered within autologous blood coagulum restores critical size segmental defects of ulna in rabbits, JBMR Plus 3 (5) (2019) e10085, , https://doi. org/10.1002/jbm4.10085. [30] S. Vukicevic, L. Grgurevic, I. Erjavec, M. Pecin, T. Bordukalo-Niksic, N. Stokovic, M. Lipar, H. Capak, D. Maticic, R. Windhager, T.K. Sampath, M. Gupta, Autologous blood coagulum is a physiological carrier for BMP6 to induce new bone formation and promote posterolateral lumbar spine fusion in rabbits, J. Tissue Eng. Regen. Med. 14 (2020) 147-115. 6. Conclusion BMPs were purified from bovine bone extracts employing a subcutaneous implant assay for bone induction. By utilizing the primary amino acid sequences obtained from purified bovine bone inductive proteins, numerous BMP genes were identified from human cDNA and genomic libraries. They are called BMPs, CDMPs and GDFs and constitute a large member of the TGF-beta superfamily of proteins. BMP proteins are highly conserved from fly to humans. Furthermore, they are expressed in many organs during embryogenesis and in part can be recapitulated during adult tissue repair. BMPs signal through a set of specific Ser-Thr kinase receptors and act under the influence of a concentration gradient, which is governed by extracellular matrix proteins and a family of BMP antagonists. Though recombinant BMP protein containing osteogenic devices are approved for therapeutic use in orthopedic medicine, there are numerous challenges due to the high doses employed suggesting need for a physiologically acceptable scaffold. The demonstration that autologous blood coagulum can serve as a physiological carrier for BMPs suggests that some of these challenges could be overcome in the future. Declaration of competing interest TKS is Co-Founder and CEO of perForm biologics Inc., and has ownership in the company, and AHR has no conflict of interest. Acknowledgement TKS thanks David C. 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