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James R. Lupski

The University of Texas Southwestern Medical Center · US
Area of research
Genetics · Cellular and Molecular Neuroscience
Research interest
Research interests include Genomic variations and chromosomal abnormalities, Genomics and Rare Diseases, Hereditary Neurological Disorders, and Genetics and Neurodevelopmental Disorders.
h-index
145
citations
87,831
works
1,083
NIH funding
primary concept
email

Recent publications

Author Correction: Biallelic variants in the noncoding RNA gene RNU4-2 cause a recessive neurodevelopmental syndrome with distinct white matter changes.
2026cited by 0position: contributordoi
De novo variants in the splicing factor gene SF3B1 are associated with neurodevelopmental disorders.
2026cited by 0position: contributordoi
Biallelic variants in the noncoding RNA gene RNU4-2 cause a recessive neurodevelopmental syndrome with distinct white matter changes.
2026cited by 0position: contributordoi
Autosomal dominant CDC45 deficiency with allelic expression bias causes a novel genetic disease of the immune system
2026cited by 0position: contributordoi
Building an Interoperable Rare Disease Multi-omic Resource: The GREGoR Data Model and Dataset
2026cited by 0position: contributordoi
Familial medullary thyroid carcinoma secondary to an <i>SLC30A9</i> intragenic deletion and translation reinitiation
2026cited by 0position: contributordoi
Monoallelic expression can govern penetrance of inborn errors of immunity
Nature 2025cited by 68position: middledoi
Monoallelic expression can govern penetrance of inborn errors of immunity.
2025cited by 47position: contributordoi
GREGoR: accelerating genomics for rare diseases.
2025cited by 10position: contributordoi
Biallelic variation in the choline and ethanolamine transporter FLVCR1 underlies a severe developmental disorder spectrum
Genetics in Medicine 2025cited by 10position: contributordoi
Genomic rare variant mechanisms for congenital cardiac laterality defect: A digenic model approach
The American Journal of Human Genetics 2025cited by 8position: middledoi
Genomic Balancing Act: deciphering DNA rearrangements in the complex chromosomal aberration involving 5p15.2, 2q31.1, and 18q21.32.
2025cited by 6position: contributordoi
DNA-binding affinity and specificity determine the phenotypic diversity in BCL11B-related disorders
The American Journal of Human Genetics 2025cited by 5position: middledoi
RNA methyltransferase SPOUT1/CENP-32 links mitotic spindle organization with the neurodevelopmental disorder SpADMiSS.
2025cited by 1position: contributordoi
Expanding the Clinical and Molecular Spectrum of <i>TUBB2B</i> Through Distinct Variants Identified Across Multiple Families
2025cited by 1position: contributordoi
Allele-specific correction of ATAD3A pathogenic variants via template-free CRISPR-Cas9 editing and gene conversion
2025cited by 0position: contributordoi
2024 Lifetime Achievement Award: Biology unbalanced: Genes, gene dosage, and disease susceptibility.
2025cited by 0position: contributordoi
CEP76 impairment at the centrosome-cilium interface contributes to a spectrum of ciliopathies.
2025cited by 0position: contributordoi
Pathogenic <i>DVL</i> frameshifting variants in Robinow syndrome disrupt WNT signaling and cellular dynamics
2025cited by 0position: contributordoi
Author response: EPHA4 signaling dysregulation links abnormal locomotion and the development of idiopathic scoliosis
2025cited by 0position: contributordoi
DE NOVO VARIANTS IN THE POLY(RC)-BINDING PROTEIN GENE <i>PCBP1</i> CAUSE A NEURODEVELOPMENTAL DISORDER
2025cited by 0position: contributordoi
Phase Separation Contributes to Pathogenicity for Nonsense Mediated Decay-Escaping Variant Alleles
2025cited by 0position: contributordoi
EPHA4 signaling dysregulation links abnormal locomotion and the development of idiopathic scoliosis
2025cited by 0position: contributordoi
Inverted triplications formed by iterative template switches generate structural variant diversity at genomic disorder loci
Cell Genomics 2024cited by 29position: contributordoi
Inverted triplications formed by iterative template switches generate structural variant diversity at genomic disorder loci
Cell Genomics 2024cited by 29position: middledoi
Individuals with <i>JAK1</i> variants are affected by syndromic features encompassing autoimmunity, atopy, colitis, and dermatitis
Journal of Experimental Medicine 2024cited by 22position: contributordoi
Individuals with <i>JAK1</i> variants are affected by syndromic features encompassing autoimmunity, atopy, colitis, and dermatitis
The Journal of Experimental Medicine 2024cited by 22position: middledoi
Using multiplexed functional data to reduce variant classification inequities in underrepresented populations
Genome Medicine 2024cited by 20position: middledoi
NODAL variants are associated with a continuum of laterality defects from simple D-transposition of the great arteries to heterotaxy.
2024cited by 16position: contributordoi
Structural variant allelic heterogeneity in MECP2 duplication syndrome provides insight into clinical severity and variability of disease expression
Genome Medicine 2024cited by 16position: middledoi

Grants

No grants ingested yet.

Frequent collaborators

· 83 papers (2019–2026)Jennifer Posey · Baylor College of Medicine37 papers (2019–2025)Richard A. Gibbs · Baylor College of Medicine34 papers (2012–2025)Donna M. Muzny · Baylor College of Medicine19 papers (2013–2020)Shalini N. Jhangiani · Baylor College of Medicine17 papers (2013–2020)Daniel G. Calame · Northwest Research Institute of Chemical Industry17 papers (2021–2025)Claudia M.B. Carvalho · Broad Institute15 papers (2012–2020)Dana Marafi · Saft (France)15 papers (2020–2024)Zeynep Coban‐Akdemir · Baylor College of Medicine11 papers (2016–2025)Chad A. Shaw · Baylor College of Medicine11 papers (2012–2018)Claudia Carvalho · National Research Centre11 papers (2019–2025)Jennifer E. Posey · Baylor College of Medicine10 papers (2015–2025)Davut Pehlivan · Kuwait University10 papers (2020–2024)Haowei Du · Green Chemistry10 papers (2020–2024) · 10 papers (2021–2024)Christopher M. Grochowski · Northwest Research Institute of Chemical Industry10 papers (2020–2024)Eric Boerwinkle · Training Programs in Epidemiology and Public Health Interventions Network9 papers (2012–2025)Tomasz Gambin · Warsaw University of Technology9 papers (2013–2018)Isabella Herman · Texas Children's Hospital9 papers (2020–2022)Nan Wu · Chongqing University9 papers (2019–2025)