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111 results on '"Hildebrandt, Friedhelm"'

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1. Steroid-Resistant Nephrotic Syndrome Is Associated With a Unique Genetic Profile in a Highly Admixed Pediatric Population

2. Pathogenic PHIP Variants are Variably Associated With CAKUT

3. Exome copy number variant detection, analysis, and classification in a large cohort of families with undiagnosed rare genetic disease

4. Implication of transcription factor FOXD2 dysfunction in syndromic congenital anomalies of the kidney and urinary tract (CAKUT)

5. The genetic landscape and clinical spectrum of nephronophthisis and related ciliopathies

8. Reverse phenotyping facilitates disease allele calling in exome sequencing of patients with CAKUT

9. Multisystem inflammation and susceptibility to viral infections in human ZNFX1 deficiency

10. Generation of Monogenic Candidate Genes for Human Nephrotic Syndrome Using 3 Independent Approaches

11. Recessive Mutations in SYNPO2 as a Candidate of Monogenic Nephrotic Syndrome

12. DAAM2 Variants Cause Nephrotic Syndrome via Actin Dysregulation

13. Phenotype expansion of heterozygous FOXC1 pathogenic variants toward involvement of congenital anomalies of the kidneys and urinary tract (CAKUT)

14. Mutations of the Transcriptional Corepressor ZMYM2 Cause Syndromic Urinary Tract Malformations

15. Whole exome sequencing identified ATP6V1C2 as a novel candidate gene for recessive distal renal tubular acidosis

18. Paralog Studies Augment Gene Discovery: DDX and DHX Genes

20. Monogenic causes of chronic kidney disease in adults

21. Mutations of ADAMTS9 Cause Nephronophthisis-Related Ciliopathy

23. A Multi-layered Quantitative In Vivo Expression Atlas of the Podocyte Unravels Kidney Disease Candidate Genes

24. Whole exome sequencing frequently detects a monogenic cause in early onset nephrolithiasis and nephrocalcinosis

25. Exome-wide Association Study Identifies GREB1L Mutations in Congenital Kidney Malformations

27. Whole exome sequencing identifies causative mutations in the majority of consanguineous or familial cases with childhood-onset increased renal echogenicity

28. A Dynamic Protein Interaction Landscape of the Human Centrosome-Cilium Interface

29. Mutations in TBX18 Cause Dominant Urinary Tract Malformations via Transcriptional Dysregulation of Ureter Development

30. DCDC2 Mutations Cause a Renal-Hepatic Ciliopathy by Disrupting Wnt Signaling

32. OXGR1 is a candidate disease gene for human calcium oxalate nephrolithiasis

35. Whole-exome resequencing reveals recessive mutations in TRAP1 in individuals with CAKUT and VACTERL association

38. Whole-exome resequencing distinguishes cystic kidney diseases from phenocopies in renal ciliopathies

39. Zebrafish Ciliopathy Screen Plus Human Mutational Analysis Identifies C21orf59 and CCDC65 Defects as Causing Primary Ciliary Dyskinesia

40. Exome Capture Reveals ZNF423 and CEP164 Mutations, Linking Renal Ciliopathies to DNA Damage Response Signaling

45. Mapping the NPHP-JBTS-MKS Protein Network Reveals Ciliopathy Disease Genes and Pathways

46. Contributors

47. De novo TRIM8 variants impair its protein localization to nuclear bodies and cause developmental delay, epilepsy, and focal segmental glomerulosclerosis

48. CC2D2A Is Mutated in Joubert Syndrome and Interacts with the Ciliopathy-Associated Basal Body Protein CEP290

50. Mutations in the Cilia Gene ARL13B Lead to the Classical Form of Joubert Syndrome

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