Research Topics
Species | Raj KapurSummaryAffiliation: University of Washington Country: USA Publications
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Publications
Transitional zone pull through: surgical pathology considerationsRaj P Kapur
Department of Laboratories, Seattle Children s Hospital, Seattle, WA 98115, USA
Semin Pediatr Surg 21:291-301. 2012..Well-controlled studies to correlate proximal margin histology, especially subtle anatomic or immunohistochemical changes, with postoperative outcome are needed...
Multiple endocrine neoplasia type 2B and Hirschsprung's diseaseRaj P Kapur
Department of Pathology, Children s Hospital and Regional Medical Center and University of Washington, Seattle, 91805, USA
Clin Gastroenterol Hepatol 3:423-31. 2005..In this review, the molecular genetics of the 2 conditions are discussed, and the clinical implications of existing data and future studies are summarized...- Calretinin immunohistochemistry versus acetylcholinesterase histochemistry in the evaluation of suction rectal biopsies for Hirschsprung DiseaseRaj P Kapur
Department of Laboratories, Chidren s Hospital and Regional Medical Center, Seattle, WA, USA
Pediatr Dev Pathol 12:6-15. 2009..Calretinin IHC appears to be a reasonable, and potentially superior, alternative to AChE as an adjunctive diagnostic method for evaluating suction rectal biopsies for HSCR... - Normal and abnormal anatomy of the cerebellar vermis in midgestational human fetusesRaj P Kapur
Department of Laboratories, Seattle Children s Hospital, Seattle, Washington 98105, USA
Birth Defects Res A Clin Mol Teratol 85:700-9. 2009.... - Gastrointestinal neuromuscular pathology in alpers diseaseRaj P Kapur
Department of Laboratories, Seattle Children s Hospital, Seattle, WA, USA
Am J Surg Pathol 35:714-22. 2011..Recognition of these distinctive light microscopic findings, in an appropriate clinical setting, should prompt the evaluation of an underlying primary mitochondriopathy... - Diffuse abnormal layering of small intestinal smooth muscle is present in patients with FLNA mutations and x-linked intestinal pseudo-obstructionRaj P Kapur
Departments of Pathology, Seattle Children s Hospital, University of Washington, WA, USA
Am J Surg Pathol 34:1528-43. 2010.... - Hox11L1 expression by precursors of enteric smooth muscle: an alternative explanation for megacecum in HOX11L1-/- miceRaj P Kapur
Department of Pathology, Children s Hospital and Regional Medical Center, 4800 Sand Point Way NE, Seattle, WA 98105, USA
Pediatr Dev Pathol 8:148-61. 2005.... - Malignant autosomal recessive osteopetrosis caused by spontaneous mutation of murine RankRaj P Kapur
Department of Pathology, Children s Hospital and Regional Medical Center, University of Washington, Seattle, Washington, USA
J Bone Miner Res 19:1689-97. 2004..The phenotype, including a block in RANKL-dependent osteoclast differentiation and lymph node agenesis, copies that of Rank(-/-) mice, which have been produced by targeted recombination... - The influence of Hox genes and three intercellular signalling pathways on enteric neuromuscular developmentR P Kapur
Department of Pathology, Children s Hospital and Regional Medical Center, Seattle, USA
Neurogastroenterol Motil 16:8-13. 2004..These signals affect enteric neural precursors as they colonize the gut, and perturbations of these molecules are associated with various types of intestinal neuropathology... - AchondrogenesisRaj P Kapur
Department of Laboratories, Children s Hospital and Regional Medical Center, Seattle, WA 98105, USA
Pediatr Dev Pathol 10:253-5. 2007..The changes reported differ from matrix alterations observed in achondrogenesis types IB or II and provide insight into the phenotypic and genotypic differences within this group of skeletal dysplasias... - Architectural malformation of the muscularis propria as a cause for intestinal pseudo-obstruction: two cases and a review of the literatureRaj P Kapur
Department of Laboratories, Children s Hospital and Regional Medical Center, Seattle, WA, USA
Pediatr Dev Pathol 12:156-64. 2009..The 2nd patient, an infant with multiple malformations, had diffuse abnormal layering of his small intestine. The rare reports of other individuals with similar pathological findings are discussed... - Practical pathology and genetics of Hirschsprung's diseaseRaj P Kapur
Department of Laboratories, Seattle Children s Hospital, University of Washington, Seattle, Washington 98115, USA
Semin Pediatr Surg 18:212-23. 2009..Many HSCR-susceptibility genes have been identified, but mutational analysis has limited practical value unless family history or clinical findings suggest syndromic HSCR... 
Recurrent reciprocal genomic rearrangements of 17q12 are associated with renal disease, diabetes, and epilepsyHeather C Mefford
Department of Genome Sciences, University of Washington, Seattle, WA 98195, USA
Am J Hum Genet 81:1057-69. 2007..We also identified the reciprocal duplication, which appears to be enriched in samples from patients with epilepsy. We describe the first example of a recurrent genomic disorder associated with diabetes...- Amelioration of enteric neuropathology in a mouse model of Niemann-Pick C by Npc1 expression in enteric gliaRaj Kapur
Department of Laboratories, Children s Hospital and Regional Medical Center, Seattle, Washington, USA
J Neurosci Res 87:2994-3001. 2009.... - Intercellular signals downstream of endothelin receptor-B mediate colonization of the large intestine by enteric neuroblastsR P Kapur
Department of Pathology, Children s Hospital, Seattle, Washington, USA
Development 121:3787-95. 1995..Instead, intercellular interactions downstream of the endothelin receptor-B mediate complete colonization of the skin and gut by neural crest cells... - Early death of neural crest cells is responsible for total enteric aganglionosis in Sox10(Dom)/Sox10(Dom) mouse embryosR P Kapur
Department of Pathology, University of Washington Medical Center, 1959 NE Pacific Street, Box 357470, Seattle, WA 98195, USA
Pediatr Dev Pathol 2:559-69. 1999..Instead, excessive cell death occurs in mutant neural crest cells early in their migratory pathway. Comparison with other models suggests that genetic heterogeneity of aganglionosis correlates with different pathogenetic mechanisms... - Transgenic rescue of aganglionosis and piebaldism in lethal spotted miceJ Rice
Department of Pathology, University of Washington, Seattle, WA 98195, USA
Dev Dyn 217:120-32. 2000..dev dyn 2000;217:120-132... - Neuropathology of paediatric chronic intestinal pseudo-obstruction and related animal modelsR P Kapur
Department of Pathology, University of Washington, Seattle, Washington 98195, USA
J Pathol 194:277-88. 2001..Information gleaned from studies of these animals is likely to change diagnostic and therapeutic approaches to paediatric CIP and related conditions... - Holoprosencephaly and limb reduction defects: a consideration of Steinfeld syndrome and related conditionsJoseph R Siebert
Department of Laboratories, Children s Hospital and Regional Medical Center, Seattle, Washington 98105, USA
Am J Med Genet A 134:381-92. 2005..The phenotypic heterogeneity inherent in these patients continues to complicate diagnosis, which will hopefully be simplified by continuing delineation at morphologic and especially genetic levels... - Hydrocephalus and intestinal aganglionosis: is L1CAM a modifier gene in Hirschsprung disease?Melissa A Parisi
Division of Genetics and Development, Department of Pediatrics, University of Washington and Children s Hospital and Regional Medical Center, Seattle, Washington 98105, USA
Am J Med Genet 108:51-6. 2002.... - Visceral neuropathy and intestinal pseudo-obstruction in a murine model of a nuclear inclusion diseaseChristine M Clarke
Department of Laboratories, Children s Hospital and Regional Medical Center, Seattle, Washington, USA
Gastroenterology 133:1971-8. 2007..The enteric neuropathology of PrP-SCA7-92Q mice was investigated after observing that they develop signs of intestinal pseudo-obstruction... - Five cases of caudal regression with an aberrant abdominal umbilical artery: Further support for a caudal regression-sirenomelia spectrumSara M Duesterhoeft
Department of Laboratories, Children s Hospital and Regional Medical Center, Seattle, Washington, USA
Am J Med Genet A 143:3175-84. 2007.... - Neuronal dysplasia: a controversial pathological correlate of intestinal pseudo-obstructionRaj P Kapur
Department of Laboratories, Children s Hospital and Regional Medical Center, University of Washington, Seattle, Washington 98105, USA
Am J Med Genet A 122:287-93. 2003..Possible genetic and developmental bases for IND are also discussed... - Genetic background modifies intestinal pseudo-obstruction and the expression of a reporter gene in Hox11L1-/- miceMelissa A Parisi
Department of Pediatrics, University of Washington School of Medicine, Children s Hospital and Regional Medical Center, 4800 Sand Point Way NW, Seattle, WA 98105, USA
Gastroenterology 125:1428-40. 2003..We investigated the regulation of Hox11L1 and factors that influence the penetrance of pseudo-obstruction in Hox11L1-null mice... - Inhibition of protein kinase A in murine enteric neurons causes lethal intestinal pseudo-obstructionDouglas G Howe
Department of Pharmacology, University of Washington, Seattle, Washington 98195 7750, USA
J Neurobiol 66:256-72. 2006.... - Association of cloacal anomalies, caudal duplication, and twinningJoseph R Siebert
Department of Laboratories A 6901, Children s Hospital and Regional Medical Center, Seattle, WA 98105, USA
Pediatr Dev Pathol 8:339-54. 2005.... - Evaluation of Hox11L1 in the fmc/fmc rat model of chronic intestinal pseudo-obstructionMelissa A Parisi
Department of Pediatrics, Children s Hospital and Regional Medical Center and University of Washington, Seattle, WA 98105, USA
J Pediatr Surg 40:1760-5. 2005..We hypothesized that fmc is a mutant allele of the rat Hox11L1 gene and tested this hypothesis by direct sequencing... - Both CD1d antigen presentation and interleukin-12 are required to activate natural killer T cells during Trypanosoma cruzi infectionMalcolm S Duthie
Infectious Disease Research Institute, 1124 Columbia St, Suite 600, Seattle, WA 98104, USA
Infect Immun 73:1890-4. 2005..The required IL-12 arises independently of MyD88. The data support a model of normal NKT-cell activation that requires IL-12 and TCR stimulation... - Critical proinflammatory and anti-inflammatory functions of different subsets of CD1d-restricted natural killer T cells during Trypanosoma cruzi infectionMalcolm S Duthie
IDRI, 1124 Columbia St, Ste 600, Seattle, WA 98104, USA
Infect Immun 73:181-92. 2005..Strikingly, most Jalpha18(-/-) mice die. Thus, in response to the same infection, vNKT cells appear to augment a robust proinflammatory response, whereas the iNKT cells dampen this response, possibly by regulating vNKT cells... - Rulers rule: present and future applications of cerebellar morphometryJoseph R Siebert
Department of Laboratories, Children's Hospital and Regional Medical Center, Seattle, WA, USA
Pediatr Dev Pathol 5:422-4. 2002 - A report of dizygous monochorionic twinsVivienne L Souter
Division of Medical Genetics, Department of Medicine, University of Washington, Seattle, USA
N Engl J Med 349:154-8. 2003 - Cytokine polymorphisms and histologic expression in autopsy studies: contribution of TNF-alpha and TGF-beta 1 to the pathogenesis of autoimmune-associated congenital heart blockRobert M Clancy
Hospital for Joint Diseases, New York University School of Medicine, Department of Rheumatology, New York, NY 10003, USA
J Immunol 171:3253-61. 2003..This profibrosing cytokine and its secretion/activation circuitry may provide a novel direction for evaluating fetal factors in the development of a robust animal model of CHB as well as therapeutic strategies in humans... - Mice with mitochondrial complex I deficiency develop a fatal encephalomyopathyShane E Kruse
Howard Hughes Medical Institute and Department of Biochemistry, University of Washington, Seattle, WA 98195, USA
Cell Metab 7:312-20. 2008..Nonetheless, total oxygen consumption and muscle ATP and phosphocreatine concentrations measured in vivo were within normal parameters... - Can we stop looking? Immunohistochemistry and the diagnosis of Hirschsprung diseaseRaj P Kapur
Am J Clin Pathol 126:9-12. 2006 - Hepatic mesenchymal hamartoma: a disorder of imprintingRobyn C Reed
Department of Laboratories, Seattle Children's Hospital and Regional Medical Center, Seattle, WA 98105, USA
Pediatr Dev Pathol 11:264-5. 2008 - Does bcl2 immunostaining really help with pathological evaluation of intestinal pseudo-obstruction?Raj P Kapur
Am J Surg Pathol 30:1059; author reply 1059-62. 2006 - A case of true hermaphroditism reveals an unusual mechanism of twinningVivienne L Souter
Department of Obstetrics and Gynecology, Banner Good Samaritan Medical Center, Phoenix, AZ 85006, USA
Hum Genet 121:179-85. 2007..The twins are chimeric and share a single genetic contribution from their mother but have two genetic contributions from their father thus supporting the existence of a third, previously unreported type of twinning... - Characterization of developing bowel transplanted from transgenic erythropoietin receptor-deficient mouse embryosMarcella Mascher-Denen
Department of Pediatrics, University of Washington, WA 98195, USA
Neonatology 93:56-63. 2008..Transgenic mice with absent Epo function are embryonic lethal, so it is not known whether Epo function is required for bowel development... - Genetic disruption of dopamine production results in pituitary adenomas and severe prolactinemiaThomas S Hnasko
Graduate Program in Neurobiology and Behavior, Children s Hospital and Regional Medical Center, University of Washington, Seattle, WA 98195, USA
Neuroendocrinology 86:48-57. 2007..Dopamine release from tuberoinfundibular dopamine neurons into the median eminence activates dopamine-D2 receptors in the pituitary gland where it inhibits lactotroph function...
Research Grants
- SACRAL CREST CELLS AND ENTERIC NEURODEVELOPMENTRaj Kapur; Fiscal Year: 2005..The results of this study will further our understanding of normal enteric neurodevelopment and congenital disorders of intestinal motility. ..