Combination of common mtDNA variants results in mitochondrial dysfunction and a connective tissue dysregulation.

connective tissue disorder histamine signaling mitochondrial disorder mtDNA haplogroups

Journal

Proceedings of the National Academy of Sciences of the United States of America
ISSN: 1091-6490
Titre abrégé: Proc Natl Acad Sci U S A
Pays: United States
ID NLM: 7505876

Informations de publication

Date de publication:
08 Nov 2022
Historique:
entrez: 2 11 2022
pubmed: 3 11 2022
medline: 5 11 2022
Statut: ppublish

Résumé

Mitochondrial dysfunction can be associated with a range of clinical manifestations. Here, we report a family with a complex phenotype including combinations of connective tissue, neurological, and metabolic symptoms that were passed on to all surviving children. Analysis of the maternally inherited mtDNA revealed a novel genotype encompassing the haplogroup J - defining mitochondrial DNA (mtDNA)

Identifiants

pubmed: 36322731
doi: 10.1073/pnas.2212417119
pmc: PMC9659340
doi:

Substances chimiques

DNA, Mitochondrial 0
Histamine 820484N8I3

Types de publication

Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, Non-P.H.S. Research Support, N.I.H., Extramural

Langues

eng

Sous-ensembles de citation

IM

Pagination

e2212417119

Subventions

Organisme : NCI NIH HHS
ID : P01 CA196539
Pays : United States
Organisme : NIAID NIH HHS
ID : R01 AI118891
Pays : United States
Organisme : HHS | National Institutes of Health (NIH)
ID : OD010944
Organisme : HHS | National Institutes of Health (NIH)
ID : MH108592

Commentaires et corrections

Type : CommentIn

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Auteurs

Patrick M Schaefer (PM)

Center for Mitochondrial and Epigenomic Medicine, Children's Hospital of Philadelphia, Philadelphia, PA 19104.

Leonardo Scherer Alves (L)

Center for Mitochondrial and Epigenomic Medicine, Children's Hospital of Philadelphia, Philadelphia, PA 19104.

Maria Lvova (M)

Center for Mitochondrial and Epigenomic Medicine, Children's Hospital of Philadelphia, Philadelphia, PA 19104.

Jessica Huang (J)

Center for Mitochondrial and Epigenomic Medicine, Children's Hospital of Philadelphia, Philadelphia, PA 19104.

Komal Rathi (K)

Department of Biomedical Informatics, Children's Hospital of Philadelphia, Philadelphia, PA 19104.

Kevin Janssen (K)

Center for Mitochondrial and Epigenomic Medicine, Children's Hospital of Philadelphia, Philadelphia, PA 19104.

Arrienne Butic (A)

Center for Mitochondrial and Epigenomic Medicine, Children's Hospital of Philadelphia, Philadelphia, PA 19104.

Tal Yardeni (T)

Center for Mitochondrial and Epigenomic Medicine, Children's Hospital of Philadelphia, Philadelphia, PA 19104.
The Bert Strassburger Metabolic Center, Sheba Medical Center, Ramat Gan 52561, Israel.

Ryan Morrow (R)

Center for Mitochondrial and Epigenomic Medicine, Children's Hospital of Philadelphia, Philadelphia, PA 19104.

Marie Lott (M)

Center for Mitochondrial and Epigenomic Medicine, Children's Hospital of Philadelphia, Philadelphia, PA 19104.

Deborah Murdock (D)

Center for Mitochondrial and Epigenomic Medicine, Children's Hospital of Philadelphia, Philadelphia, PA 19104.

Angela Song (A)

Center for Mitochondrial and Epigenomic Medicine, Children's Hospital of Philadelphia, Philadelphia, PA 19104.

Kierstin Keller (K)

Center for Mitochondrial and Epigenomic Medicine, Children's Hospital of Philadelphia, Philadelphia, PA 19104.

Benjamin A Garcia (BA)

Department of Biochemistry and Molecular Biophysics, Washington University School of Medicine, St. Louis, MO 63110.

Clair A Francomano (CA)

Department of Medical and Molecular Genetics, Indiana University School of Medicine, Indianapolis, IN 46202.

Douglas C Wallace (DC)

Center for Mitochondrial and Epigenomic Medicine, Children's Hospital of Philadelphia, Philadelphia, PA 19104.
Department of Pediatrics, Division of Human Genetics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104.

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Classifications MeSH