D. Wade Clapp
Indiana University
HaematopoiesisMolecular biologyProgenitor cellImmunologyBiology
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Publications 145
#1Katherine J. Barrett (Duke University)H-Index: 1
#2T. Michelle Cooley (Duke University)
Last. Sallie R. Permar (Duke University)H-Index: 29
view all 6 authors...
#1Yun Ma (BCM: Baylor College of Medicine)H-Index: 2
#2Andrea M. GrossH-Index: 4
Last. D. Wade Clapp (IU: Indiana University)H-Index: 38
view all 14 authors...
Plexiform neurofibromas (pNF) develop in children with neurofibromatosis type 1 (NF1) and can be associated with several skeletal comorbidities. Preclinical mouse studies revealed Nf1 deficiency in osteoprogenitor cells disrupts, in a MEK-dependent manner, pyrophosphate (PPi) homeostasis and skeletal mineralization. The etiology of NF-associated skeletal manifestations remains unknown. We used mouse models of NF1 neurofibromas to assess bone mineralization of skeletal structures adjacent to tumo...
#1Julie A. Mund (IU: Indiana University)H-Index: 13
#2Su Jung Park (IU: Indiana University)H-Index: 17
Last. Jin Yuan (IU: Indiana University)H-Index: 5
view all 16 authors...
Neurofibromatosis type 1 (NF1) is a common cancer predisposition syndrome caused by mutations in the NF1 tumor suppressor gene. NF1 encodes neurofibromin, a GTPase-activating protein (GAP) for RAS proto-oncogene GTPase (RAS). Plexiform neurofibromas (PNs) are a hallmark of NF1 and result from loss of heterozygosity of NF1 in Schwann cells, leading to constitutively activated p21RAS. Given the inability to target p21RAS directly, here we performed an shRNA library screen of all human kinases and ...
#1Amy E. Armstrong (WashU: Washington University in St. Louis)
#2Steven D. Rhodes (Riley Hospital for Children)
Last. Xianlin YangH-Index: 10
view all 15 authors...
BACKGROUND Neurofibromatosis type 1 (NF1) is a common genetic disorder characterized by plexiform neurofibromas (pNF), which are thought to be congenital tumors that arise in utero and enlarge throughout life. Genetic studies in murine models delineated an indispensable role for the stem cell factor (SCF)/c-kit pathway in pNF initiation and progression. A subsequent phase 2 clinical trial using imatinib mesylate to inhibit SCF/c-kit demonstrated tumor shrinkage in a subset of preexisting pNF; ho...
#1Fenil Shah (IUPUI: Indiana University – Purdue University Indianapolis)
#2Olivia Babb (IUPUI: Indiana University – Purdue University Indianapolis)
Last. Melissa L. Fishel (IUPUI: Indiana University – Purdue University Indianapolis)H-Index: 25
view all 14 authors...
Malignant Peripheral Nerve Sheath Tumor (MPNST) is a rare soft tissue sarcoma that can arise from patients with NF1 (neurofibromatosis type 1). These patients are at a much greater risk of developing MPNST than the general population (10% vs. 0.01%, respectively). Existing chemotherapeutic and targeted agents have thus far not been successful in MPNST treatment, with a 5-year patient survival rate of just 35%-50%. Recent research implicates Signal Transducer and Activator of Transcription-3 (STA...
#1Scott R. Plotkin (Harvard University)H-Index: 40
#2Dan G. Duda (Harvard University)H-Index: 63
Last. Matthias A. Karajannis (NYU: New York University)H-Index: 33
view all 16 authors...
PURPOSEBevacizumab treatment at 7.5 mg/kg every 3 weeks results in improved hearing in approximately 35%-40% of patients with neurofibromatosis type 2 (NF2) and progressive vestibular schwannomas (...
3 CitationsSource
#1Smita Bhatia (UAB: University of Alabama at Birmingham)H-Index: 69
#2Yanjun Chen (UAB: University of Alabama at Birmingham)H-Index: 8
Last. Michael Fisher (Children's Hospital of Philadelphia)H-Index: 51
view all 23 authors...
PURPOSEFundamental gaps in knowledge regarding the risk of subsequent neoplasms (SNs) in children with pathogenic neurofibromatosis type 1 (NF1) variants exposed to radiation and/or alkylator chemo...
3 CitationsSource
#1Steven D. Rhodes (IU: Indiana University)H-Index: 9
#2Yongzheng He (IU: Indiana University)H-Index: 9
Last. D. Wade Clapp (IU: Indiana University)H-Index: 38
view all 14 authors...
: Plexiform neurofibroma (PN) tumors are a hallmark manifestation of neurofibromatosis type 1 (NF1) that arise in the Schwann cell (SC) lineage. NF1 is a common heritable cancer predisposition syndrome caused by germline mutations in the NF1 tumor suppressor, which encodes a GTPase-activating protein called neurofibromin that negatively regulates Ras proteins. Whereas most PN are clinically indolent, a subset progress to atypical neurofibromatous neoplasms of uncertain biologic potential (ANNUBP...
9 CitationsSource
#1Renyuan Bai (JHUSOM: Johns Hopkins University School of Medicine)H-Index: 11
#1Ren Yuan Bai (JHUSOM: Johns Hopkins University School of Medicine)H-Index: 5
Last. Verena Staedtke (JHUSOM: Johns Hopkins University School of Medicine)H-Index: 12
view all 7 authors...
Neurofibromatosis type 1, including the highly aggressive malignant peripheral nerve sheath tumors (MPNSTs), is featured by the loss of functional neurofibromin 1 (NF1) protein resulting from genetic alterations. A major function of NF1 is suppressing Ras activities, which is conveyed by an intrinsic GTPase-activating protein-related domain (GRD). In this study, we explored the feasibility of restoring Ras GTPase via exogenous expression of various GRD constructs, via gene delivery using a panel...
5 CitationsSource
#1Jeffrey R Gehlhausen (IU: Indiana University)H-Index: 1
#2Eric T. Hawley (IU: Indiana University)H-Index: 2
Last. Su Jung Park (IU: Indiana University)H-Index: 17
view all 24 authors...
: Schwannomas are common, highly morbid and medically untreatable tumors that can arise in patients with germ line as well as somatic mutations in neurofibromatosis type 2 (NF2). These mutations most commonly result in the loss of function of the NF2-encoded protein, Merlin. Little is known about how Merlin functions endogenously as a tumor suppressor and how its loss leads to oncogenic transformation in Schwann cells (SCs). Here, we identify nuclear factor kappa-light-chain-enhancer of activate...
2 CitationsSource