Spectrum of

human genetics molecular medicine mutation neurodegenerative diseases neurology

Journal

Journal of medical genetics
ISSN: 1468-6244
Titre abrégé: J Med Genet
Pays: England
ID NLM: 2985087R

Informations de publication

Date de publication:
03 Oct 2023
Historique:
received: 23 05 2023
accepted: 10 09 2023
medline: 4 10 2023
pubmed: 4 10 2023
entrez: 3 10 2023
Statut: aheadofprint

Résumé

Chediak-Higashi syndrome (CHS) is a rare autosomal recessive disorder characterised by partial oculocutaneous albinism, a bleeding diathesis, immunological dysfunction and neurological impairment. Bi-allelic loss-of-function variants in To further our understanding of the pathogenesis of CHS, we conducted clinical evaluations on individuals with CHS enrolled in our natural history study. Using genomic DNA Sanger sequencing, we identified novel pathogenic Our investigation unveiled 11 novel pathogenic The identification of novel pathogenic

Identifiants

pubmed: 37788905
pii: jmg-2023-109420
doi: 10.1136/jmg-2023-109420
pii:
doi:

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Informations de copyright

© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.

Déclaration de conflit d'intérêts

Competing interests: None declared.

Auteurs

Marie Morimoto (M)

NIH Undiagnosed Diseases Program, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA.

Elena-Raluca Nicoli (ER)

NIH Undiagnosed Diseases Program, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA.

Chulaluck Kuptanon (C)

Human Biochemical Genetics Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA.

Joseph C Roney (JC)

Human Biochemical Genetics Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA.

Jenny Serra-Vinardell (J)

Human Biochemical Genetics Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA.

Prashant Sharma (P)

NIH Undiagnosed Diseases Program, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA.

David R Adams (DR)

NIH Undiagnosed Diseases Program, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA.
Office of the Clinical Director, National Institutes of Health, Bethesda, Maryland, USA.

John I Gallin (JI)

Clinical Pathophysiology Section, Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland, USA.

Steven M Holland (SM)

Immunopathogenesis Section, Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland, USA.

Sergio D Rosenzweig (SD)

Department of Laboratory Medicine, National Institute of Allergy and Infectious Diseases, Bethesda, Maryland, USA.

Jose Barbot (J)

Unidade de Hematologia, Serviço de Pediatria, Centro Hospitalar do Porto, Porto, Portugal.

Carla Ciccone (C)

Human Biochemical Genetics Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA.

Marjan Huizing (M)

Human Biochemical Genetics Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA.

Camilo Toro (C)

NIH Undiagnosed Diseases Program, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA.

William A Gahl (WA)

NIH Undiagnosed Diseases Program, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA.
Human Biochemical Genetics Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA.

Wendy J Introne (WJ)

Human Biochemical Genetics Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA.

May Christine V Malicdan (MCV)

NIH Undiagnosed Diseases Program, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA maychristine.malicdan@nih.gov.
Human Biochemical Genetics Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA.

Classifications MeSH