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BolA family member 1 (BOLA1)
- Target
- BOLA1
- Molecular classification
- Other (member of BolA protein family), Mitochondrial protein
01
Overview
BolA family member 1 (BOLA1) is a mitochondrial protein implicated in the regulation of mitochondrial morphology and maintenance of redox homeostasis, particularly the mitochondrial thiol redox potential. It interacts with monothiol glutaredoxin GLRX5 and is part of the mitochondrial iron-sulfur (Fe-S) cluster assembly machinery, facilitating the insertion of Fe-S clusters into specific mitochondrial proteins necessary for mitochondrial function. Loss of BOLA1 function increases oxidation of mitochondrial thiol groups and may negatively affect mitochondrial dynamics. BOLA1 is primarily found in aerobic eukaryotes, and mutations or dysfunction are associated with mitochondrial disease syndromes, but it is not a direct therapeutic target or receptor as currently understood.
- Other names
- BolA-like protein 1CGI-143hBolAbolA homolog 1bolA-like 1
02
Biological functions
Mitochondrial morphology regulationMaintenance of mitochondrial thiol redox potentialRegulation of iron-sulfur (Fe-S) cluster assemblyCellular redox homeostasisIntracellular iron ion homeostasis
03
Disease associations
Mitochondrial diseaseMultiple mitochondrial dysfunctions syndrome (with hyperglycinemia)Possible link to neurodegeneration (mechanistic, not direct clinical evidence)
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