BOLA (BolA Family Member 3) Deficiency Controls Endothelial Metabolism and Glycine Homeostasis in Pulmonary Hypertension | Zendy

Qiujun Yu | Zendy; YiYin Tai | Zendy; Ying Tang | Zendy; Jingsi Zhao | Zendy; Vinny Negi | Zendy; Miranda K. Culley | Zendy; Jyotsna Pilli | Zendy; Wei Sun | Zendy; Karin Brugger | Zendy; Johannes A. Mayr | Zendy; Rajeev Saggar | Zendy; Rajan Saggar | Zendy; W. Dean Wallace | Zendy; D. J. A. Ross | Zendy; Aaron B. Waxman | Zendy; Stacy G. Wendell | Zendy; Steven J. Mullett | Zendy; John Sembrat | Zendy; Mauricio Rojas | Zendy; Omar F. Khan | Zendy; James E. Dahlman | Zendy; Masataka Sugahara | Zendy; Nobuyuki Kagiyama | Zendy; Taijyu Satoh | Zendy; Manling Zhang | Zendy; Ning Feng | Zendy; John Gorcsan | Zendy; Sara O. Vargas | Zendy; Kathleen J. Haley | Zendy; Rahul Kumar | Zendy; Brian B. Graham | Zendy; Róbert Langer | Zendy; Daniel G. Anderson | Zendy; Bing Wang | Zendy; Sruti Shiva | Zendy; Thomas Bertero | Zendy; Stephen Y. Chan | Zendy

Open Access

BOLA (BolA Family Member 3) Deficiency Controls Endothelial Metabolism and Glycine Homeostasis in Pulmonary Hypertension

Author(s) -

Qiujun Yu,

YiYin Tai,

Ying Tang,

Jingsi Zhao,

Vinny Negi,

Miranda K. Culley,

Jyotsna Pilli,

Wei Sun,

Karin Brugger,

Johannes A. Mayr,

Rajeev Saggar,

Rajan Saggar,

W. Dean Wallace,

D. J. A. Ross,

Aaron B. Waxman,

Stacy G. Wendell,

Steven J. Mullett,

John Sembrat,

Mauricio Rojas,

Omar F. Khan,

James E. Dahlman,

Masataka Sugahara,

Nobuyuki Kagiyama,

Taijyu Satoh,

Manling Zhang,

Ning Feng,

John Gorcsan,

Sara O. Vargas,

Kathleen J. Haley,

Rahul Kumar,

Brian B. Graham,

Róbert Langer,

Daniel G. Anderson,

Bing Wang,

Sruti Shiva,

Thomas Bertero,

Stephen Y. Chan

Publication year - 2019

Publication title -

circulation

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 7.795

H-Index - 607

eISSN - 1524-4539

pISSN - 0009-7322

DOI - 10.1161/circulationaha.118.035889

Subject(s) - pulmonary hypertension , hypoxic pulmonary vasoconstriction , mitochondrial biogenesis , medicine , mitochondrion , gene knockdown , microbiology and biotechnology , biology , cancer research , biochemistry , gene

Deficiencies of iron-sulfur (Fe-S) clusters, metal complexes that control redox state and mitochondrial metabolism, have been linked to pulmonary hypertension (PH), a deadly vascular disease with poorly defined molecular origins. BOLA3 (BolA Family Member 3) regulates Fe-S biogenesis, and mutations in BOLA3 result in multiple mitochondrial dysfunction syndrome, a fatal disorder associated with PH. The mechanistic role of BOLA3 in PH remains undefined.

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