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355 results on '"Jhangiani, Shalini N"'

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1. NODAL variants are associated with a continuum of laterality defects from simple D-transposition of the great arteries to heterotaxy

2. Inverted triplications formed by iterative template switches generate structural variant diversity at genomic disorder loci

3. Biallelic variation in the choline and ethanolamine transporter FLVCR1 underlies a severe developmental disorder spectrum

4. The impact of the Turkish population variome on the genomic architecture of rare disease traits

5. Monoallelic variation in DHX9, the gene encoding the DExH-box helicase DHX9, underlies neurodevelopment disorders and Charcot-Marie-Tooth disease

6. Rare variant enrichment analysis supports GREB1L as a contributory driver gene in the etiology of Mayer-Rokitansky-Küster-Hauser syndrome

7. The multiple de novo copy number variant (MdnCNV) phenomenon presents with peri-zygotic DNA mutational signatures and multilocus pathogenic variation

8. Biallelic loss-of-function variants in the splicing regulator NSRP1 cause a severe neurodevelopmental disorder with spastic cerebral palsy and epilepsy

9. IFIH1 loss-of-function variants contribute to very early-onset inflammatory bowel disease

10. Biallelic and monoallelic variants in PLXNA1 are implicated in a novel neurodevelopmental disorder with variable cerebral and eye anomalies

11. Exome sequencing reveals predominantly de novo variants in disorders with intellectual disability (ID) in the founder population of Finland

12. High prevalence of multilocus pathogenic variation in neurodevelopmental disorders in the Turkish population

14. Human NK cell deficiency as a result of biallelic mutations in MCM10

15. Disease-associated CTNNBL1 mutation impairs somatic hypermutation by decreasing nuclear AID

16. Low-level parental somatic mosaic SNVs in exomes from a large cohort of trios with diverse suspected Mendelian conditions

17. Genetic and mechanistic diversity in pediatric hemophagocytic lymphohistiocytosis

18. Insights into genetics, human biology and disease gleaned from family based genomic studies

19. Genetic architecture of laterality defects revealed by whole exome sequencing

20. Primary immunodeficiency diseases: Genomic approaches delineate heterogeneous Mendelian disorders

21. Integrated sequencing and array comparative genomic hybridization in familial Parkinson disease

22. Identification of likely pathogenic and known variants in TSPEAR, LAMB3, BCOR, and WNT10A in four Turkish families with tooth agenesis

23. A novel NAA10 variant with impaired acetyltransferase activity causes developmental delay, intellectual disability, and hypertrophic cardiomyopathy

24. Comprehensive genomic analysis of patients with disorders of cerebral cortical development

25. Biallelic variants in KIF14 cause intellectual disability with microcephaly

27. Interchromosomal template-switching as a novel molecular mechanism for imprinting perturbations associated with Temple syndrome

28. Biallelic missense variants in COG3 cause a congenital disorder of glycosylation with impairment of retrograde vesicular trafficking.

29. Novel LSS variants in alopecia and intellectual disability syndrome: New case report and clinical spectrum of LSS‐related rare disease traits.

30. Biallelic mutations in IRF8 impair human NK cell maturation and function

31. The sheep genome illuminates biology of the rumen and lipid metabolism

32. Molecular etiology of arthrogryposis in multiple families of mostly Turkish origin

33. Pathogenetics of alveolar capillary dysplasia with misalignment of pulmonary veins

34. Dual molecular diagnosis contributes to atypical Prader–Willi phenotype in monozygotic twins

35. A biallelic frameshift indel in PPP1R35 as a cause of primary microcephaly.

36. Epistasis dominates the genetic architecture of Drosophila quantitative traits

37. Germline Mutations in Shelterin Complex Genes Are Associated With Familial Glioma

39. Compound Heterozygous CORO1A Mutations in Siblings with a Mucocutaneous-Immunodeficiency Syndrome of Epidermodysplasia Verruciformis-HPV, Molluscum Contagiosum and Granulomatous Tuberculoid Leprosy

41. Hemichordate genomes and deuterostome origins

44. Homozygous Loss-of-function Mutations in SOHLH1 in Patients With Nonsyndromic Hypergonadotropic Hypogonadism

45. FBN1 contributing to familial congenital diaphragmatic hernia

48. The Drosophila melanogaster Genetic Reference Panel

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