Bernard L Maria

Summary

Affiliation: Medical University of South Carolina
Country: USA

Publications

  1. pmc Targeting hyaluronan interactions in spinal cord astrocytomas and diffuse pontine gliomas
    Bernard L Maria
    Department of Pediatrics, Charles P Darby Children s Research Institute, College of Medicine, Medical University of South Carolina, Charleston, South Carolina 29425, USA
    J Child Neurol 23:1214-20. 2008
  2. ncbi request reprint Tuberous sclerosis complex: pathogenesis, diagnosis, strategies, therapies, and future research directions
    Bernard L Maria
    Children s Research Institute, Medical University of South Carolina, Charleston, SC 29425, USA
    J Child Neurol 19:632-42. 2004
  3. pmc Hyaluronan regulates ceruloplasmin production by gliomas and their treatment-resistant multipotent progenitors
    Sandra L Tye
    Department of Pediatrics, Charles P Darby Children s Research Institute, College of Medicine, Medical University of South Carolina, South Carolina 29425, USA
    J Child Neurol 23:1221-30. 2008
  4. doi request reprint Targeting hyaluronan interactions in malignant gliomas and their drug-resistant multipotent progenitors
    Anne G Gilg
    Department of Pediatrics, Charles P Darby Children s Research Institute, Medical University of South Carolina, Charleston, SC 29425, USA
    Clin Cancer Res 14:1804-13. 2008
  5. pmc CD147-dependent heterogeneity in malignant and chemoresistant properties of cancer cells
    Lu Dai
    Department of Regenerative Medicine and Cell Biology, Medical University of South Carolina, Charleston, South Carolina 29425, USA
    Am J Pathol 182:577-85. 2013
  6. pmc Hyaluronan, CD44, and emmprin regulate lactate efflux and membrane localization of monocarboxylate transporters in human breast carcinoma cells
    Mark G Slomiany
    Departments of Cell Biology and Anatomy, Medical University of South Carolina, Charleston, South Carolina 29425, USA
    Cancer Res 69:1293-301. 2009
  7. pmc Bone loss in survival motor neuron (Smn(-/-) SMN2) genetic mouse model of spinal muscular atrophy
    Srinivasan Shanmugarajan
    Charles P Darby Children s Research Institute, Charleston, SC 29425, USA
    J Pathol 219:52-60. 2009
  8. pmc Abrogating drug resistance in malignant peripheral nerve sheath tumors by disrupting hyaluronan-CD44 interactions with small hyaluronan oligosaccharides
    Mark G Slomiany
    Department of Cell Biology and Anatomy, Medical University of South Carolina, Charleston, South Carolina 29425, USA
    Cancer Res 69:4992-8. 2009
  9. pmc Tumors of the central nervous system: clinical aspects, molecular mechanisms, unanswered questions, and future research directions
    Michael A Babcock
    College of Medicine, Medical University of South Carolina, Charleston, South Carolina 29425, USA
    J Child Neurol 23:1103-21. 2008
  10. ncbi request reprint Acute disseminated encephalomyelitis following infectious mononucleosis
    Hamid R Bahadori
    Medical University of South Carolina, Charleston, SC 29425, USA
    J Child Neurol 22:324-8. 2007

Research Grants

  1. NEUROBIOLOGY OF DISEASE IN CHILDREN CONFERENCES
    Bernard Maria; Fiscal Year: 2007

Collaborators

Detail Information

Publications29

  1. pmc Targeting hyaluronan interactions in spinal cord astrocytomas and diffuse pontine gliomas
    Bernard L Maria
    Department of Pediatrics, Charles P Darby Children s Research Institute, College of Medicine, Medical University of South Carolina, Charleston, South Carolina 29425, USA
    J Child Neurol 23:1214-20. 2008
    ..Furthermore, we describe novel animal models of these tumors for preclinical studies. These findings suggest that hyaluronan antagonism has potential therapeutic value in malignant central nervous system tumors...
  2. ncbi request reprint Tuberous sclerosis complex: pathogenesis, diagnosis, strategies, therapies, and future research directions
    Bernard L Maria
    Children s Research Institute, Medical University of South Carolina, Charleston, SC 29425, USA
    J Child Neurol 19:632-42. 2004
  3. pmc Hyaluronan regulates ceruloplasmin production by gliomas and their treatment-resistant multipotent progenitors
    Sandra L Tye
    Department of Pediatrics, Charles P Darby Children s Research Institute, College of Medicine, Medical University of South Carolina, South Carolina 29425, USA
    J Child Neurol 23:1221-30. 2008
    ..Further gain and loss-of-function studies are needed to fully define the relationship between hyaluronan and ceruloplasmin, and ceruloplasmin's effect on malignant behaviors...
  4. doi request reprint Targeting hyaluronan interactions in malignant gliomas and their drug-resistant multipotent progenitors
    Anne G Gilg
    Department of Pediatrics, Charles P Darby Children s Research Institute, Medical University of South Carolina, Charleston, SC 29425, USA
    Clin Cancer Res 14:1804-13. 2008
    ..To determine if hyaluronan oligomers (o-HA) antagonize the malignant properties of glioma cells and treatment-resistant glioma side population (SP) cells in vitro and in vivo...
  5. pmc CD147-dependent heterogeneity in malignant and chemoresistant properties of cancer cells
    Lu Dai
    Department of Regenerative Medicine and Cell Biology, Medical University of South Carolina, Charleston, South Carolina 29425, USA
    Am J Pathol 182:577-85. 2013
    ..These results show that CD147 is associated with assembly of numerous pro-oncogenic proteins in the plasma membrane and may play a fundamental role in properties characteristic of cancer stem-like cells...
  6. pmc Hyaluronan, CD44, and emmprin regulate lactate efflux and membrane localization of monocarboxylate transporters in human breast carcinoma cells
    Mark G Slomiany
    Departments of Cell Biology and Anatomy, Medical University of South Carolina, Charleston, South Carolina 29425, USA
    Cancer Res 69:1293-301. 2009
    ..Together, these data indicate that constitutive interactions among hyaluronan, CD44, and emmprin contribute to regulation of MCT localization and function in the plasma membrane of breast carcinoma cells...
  7. pmc Bone loss in survival motor neuron (Smn(-/-) SMN2) genetic mouse model of spinal muscular atrophy
    Srinivasan Shanmugarajan
    Charles P Darby Children s Research Institute, Charleston, SC 29425, USA
    J Pathol 219:52-60. 2009
    ..Our results implicate SMN function in bone remodelling and skeletal pathogenesis in SMA. Understanding basic mechanisms of SMN action in bone remodelling may uncover new therapeutic targets for preventing bone loss/fracture risk in SMA...
  8. pmc Abrogating drug resistance in malignant peripheral nerve sheath tumors by disrupting hyaluronan-CD44 interactions with small hyaluronan oligosaccharides
    Mark G Slomiany
    Department of Cell Biology and Anatomy, Medical University of South Carolina, Charleston, South Carolina 29425, USA
    Cancer Res 69:4992-8. 2009
    ..These results also show the potential efficacy of hyaluronan oligomers, which are nontoxic and nonimmunogenic, as an adjuvant for chemotherapy in MPNST patients...
  9. pmc Tumors of the central nervous system: clinical aspects, molecular mechanisms, unanswered questions, and future research directions
    Michael A Babcock
    College of Medicine, Medical University of South Carolina, Charleston, South Carolina 29425, USA
    J Child Neurol 23:1103-21. 2008
    ..A cooperative effort is crucial to early diagnosis and to translating basic research findings into safe, effective new treatments...
  10. ncbi request reprint Acute disseminated encephalomyelitis following infectious mononucleosis
    Hamid R Bahadori
    Medical University of South Carolina, Charleston, SC 29425, USA
    J Child Neurol 22:324-8. 2007
    ..Although Epstein-Barr virus can cause acute viral encephalomyelitis, the authors report a case of acute disseminated encephalomyelitis months after acute Epstein-Barr virus infection...
  11. doi request reprint Absence of decussation of the superior cerebellar peduncles in patients with Joubert syndrome
    M Vittoria Spampinato
    Radiology Department, Medical University of South Carolina, Charleston, South Carolina 29425, USA
    Am J Med Genet A 146:1389-94. 2008
    ....
  12. doi request reprint Neurofibromatosis type 1 revisited
    Virginia C Williams
    Department of Pediatrics, Medical University of South Carolina, Charleston, SC 29425, USA
    Pediatrics 123:124-33. 2009
    ..In this review, we describe the clinical manifestations, recent molecular and genetic findings, and current and developing therapies for managing clinical problems associated with neurofibromatosis type 1...
  13. pmc Injury to the preterm brain and cerebral palsy: clinical aspects, molecular mechanisms, unanswered questions, and future research directions
    Michael A Babcock
    College of Medicine, Medical University of South Carolina, Charleston, South Carolina, USA
    J Child Neurol 24:1064-84. 2009
    ..A cooperative effort is necessary to translate basic research findings into clinically effective therapies and better care for these children...
  14. doi request reprint Evidence-based versus reported epilepsy management practices
    Katherine S Mastriani
    Division of Pediatric Epidemiology, Medical University of South Carolina, Charleston, SC, USA
    J Child Neurol 23:507-14. 2008
    ..Safety and efficacy data suggest that the ketogenic diet should be more widely adopted as a management strategy in pediatric epilepsy...
  15. pmc Congenital bone fractures in spinal muscular atrophy: functional role for SMN protein in bone remodeling
    Srinivasan Shanmugarajan
    Charles P Darby Children s Research Institute, Medical University of South Carolina, Charleston, South Carolina 29425, USA
    J Child Neurol 22:967-73. 2007
    ..This article reviews the skeletal complications associated with spinal muscular atrophy and describes a functional role for SMN protein in osteoclast development and bone resorption activity...
  16. ncbi request reprint Spinal muscular atrophy: classification, diagnosis, management, pathogenesis, and future research directions
    Felina V Kostova
    Medical University of South Carolina, Charleston, South Carolina, USA
    J Child Neurol 22:926-45. 2007
    ..This article summarizes the presentations and includes the verbatim edited transcript of question-and-answer sessions...
  17. ncbi request reprint Cerebral and cerebellar motor activation abnormalities in a subject with Joubert syndrome: functional magnetic resonance imaging (MRI) study
    Melissa A Parisi
    Department of Pediatrics, University of Washington, School of Medicine, Seattle 98195, USA
    J Child Neurol 19:214-8. 2004
    ..Malformation of the hindbrain could result in recruitment of alternative pathways, similar to what has been observed following ischemic injury to the developing or mature central nervous system...
  18. pmc Childhood ataxia: clinical features, pathogenesis, key unanswered questions, and future directions
    Claire N Ashley
    Department of Pediatrics, Medical College of Georgia, Georgia Health Sciences University, Augusta, GA 30912, USA
    J Child Neurol 27:1095-120. 2012
    ....
  19. pmc Modulation of tumor tolerance in primary central nervous system malignancies
    Theodore S Johnson
    Department of Pediatrics, Georgia Health Sciences University, 1120 Fifteenth Street, Augusta, GA 30912, USA
    Clin Dev Immunol 2012:937253. 2012
    ..Thus, it is an open question whether tumor tolerance in the brain is fundamentally different from that of peripheral sites of tumorigenesis or whether it simply stands as a particularly strong example of such tolerance...
  20. ncbi request reprint Primary antiphospholipid syndrome presenting with a branch retinal artery occlusion in a 15-year-old boy
    Matthew A Saxonhouse
    Department of Pediatrics, Shands Children s Hospital, University of Florida, Gainesville 32610 0296, USA
    J Child Neurol 17:392-4. 2002
    ..Complete evaluation revealed significant elevations in antiphospholipid antibodies. To our knowledge, there are no cases in children of primary antiphospholipid syndrome presenting with this clinical manifestation...
  21. ncbi request reprint Tuberous sclerosis complex: future research directions
    Bernard L Maria
    J Child Neurol 19:631. 2004
  22. ncbi request reprint Oromotor and communication findings in joubert syndrome: further evidence of multisystem apraxia
    Barbara A Braddock
    Department of Psychological Sciences, University of Missouri Columbia, USA
    J Child Neurol 21:160-3. 2006
    ....
  23. ncbi request reprint Sturge-Weber syndrome: a review
    Kristin A Thomas-Sohl
    Department of Child Health, University of Missouri Columbia, Missouri, USA
    Pediatr Neurol 30:303-10. 2004
    ..In this review, we describe the syndrome's characteristic features, clinical course, and optimal management...
  24. ncbi request reprint Parenting stress and its relationship to the behavior of children with Joubert syndrome
    Janet E Farmer
    Department of Health Psychology, University of Missouri Columbia, USA
    J Child Neurol 21:163-7. 2006
    ..Findings provide a better understanding of the impact of child behavior on parents caring for a child with Joubert syndrome...
  25. ncbi request reprint Molar tooth sign of the midbrain-hindbrain junction: occurrence in multiple distinct syndromes
    Joseph G Gleeson
    Division of Pediatric Neurology, Department of Neurosciences, University of California, San Diego, California 92093 0624, USA
    Am J Med Genet A 125:125-34; discussion 117. 2004
    ..Proper classification of patients with these variants of the MTS will be essential for localization and identification of mutant genes...
  26. ncbi request reprint Ex vivo high-resolution magnetic resonance imaging of the brain in Joubert's syndrome
    Kyle R Padgett
    McKnight Brain Institute, Department of Nuclear and Radiological Sciences, University of Florida, Gainesville, USA
    J Child Neurol 17:911-3. 2002
    ..The results of this study suggest that high-resolution MRI may be useful in screening the brainstem for malformations that can be studied histologically in a much more targeted fashion...
  27. ncbi request reprint The Neurobiology of Disease in Children Conference
    Bernard L Maria
    J Child Neurol 22:923-5. 2007
  28. pmc RPGRIP1L mutations are mainly associated with the cerebello-renal phenotype of Joubert syndrome-related disorders
    F Brancati
    Istituto di Ricovero e Cura a Carattere Scientifico, CSS Mendel Institute, Rome, Italy
    Clin Genet 74:164-70. 2008
    ..Conversely, no pathogenic changes were found in patients with other JSRD phenotypes, suggesting that RPGRIP1L mutations are largely confined to the cerebello-renal subgroup, while they overall represent a rare cause of JSRD (<2%)...
  29. ncbi request reprint The face of Joubert syndrome: a study of dysmorphology and anthropometry
    Stephen R Braddock
    University of Virginia Pediatrics, Charlottesville, California, USA
    Am J Med Genet A 143:3235-42. 2007
    ..Variability and overlap of features in JS and other hindbrain syndromes makes clinical diagnosis difficult and probably reflects genetic heterogeneity within the cohort of patients with the MTS...

Research Grants2

  1. NEUROBIOLOGY OF DISEASE IN CHILDREN CONFERENCES
    Bernard Maria; Fiscal Year: 2007
    ....