- Tel: 858.663.9055
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Email: info@nsjbio.com
- Tel: 858.663.9055
- Email: info@nsjbio.com
BOLA3 Antibody PCRP-BOLA3-1A5 / BolA-like Protein 3 Monoclonal Antibody [clone PCRP-BOLA3-1A5] (V4033)
Email: info@nsjbio.com
| Catalog No | Formulation | Size | Price (USD) | ||
|---|---|---|---|---|---|
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V4033-100UG | 0.2 mg/ml in 1X PBS with 0.1 mg/ml BSA (US sourced), 0.05% sodium azide | 100 ug | 559 | |
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V4033-20UG | 0.2 mg/ml in 1X PBS with 0.1 mg/ml BSA (US sourced), 0.05% sodium azide | 20 ug | 259 | |
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V4033SAF-100UG | 1 mg/ml in 1X PBS; BSA free, sodium azide free | 100 ug | 559 |
| Species Reactivity | Human |
| Format | Purified |
| Host | Mouse |
| Clonality | Monoclonal (mouse origin) |
| Isotype | Mouse IgG1 |
| Clone Name | PCRP-BOLA3-1A5 |
| Purity | Protein A/G affinity |
| UniProt | Q53S33 |
| Localization | Nucleus, cytoplasm |
| Applications | Flow Cytometry : 1-2ug/million cells |
| Limitations | This BOLA3 Antibody PCRP-BOLA3-1A5 / BolA-like Protein 3 Monoclonal Antibody is available for research use only. |
|
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BOLA3 Antibody PCRP-BOLA3-1A5 / BolA-like Protein 3 Monoclonal Antibody recognizes BOLA3, a mitochondrial protein involved in the maturation of iron-sulfur (Fe-S) cluster-containing proteins. Encoded by the BOLA3 gene, this protein belongs to the conserved BolA family and contributes to mitochondrial cofactor assembly through interactions with proteins involved in Fe-S cluster trafficking and transfer. These cofactors are essential for numerous metabolic enzymes, including components of the mitochondrial respiratory chain and enzymes participating in intermediary metabolism.
BOLA3 functions in the mitochondrial matrix, where it participates in the late stages of Fe-S protein maturation. Its activity is associated with the transfer and insertion of Fe-S clusters into selected mitochondrial client proteins. BOLA3 interacts functionally with proteins such as NFU1 and glutaredoxin 5 (GLRX5), which contribute to the organization and delivery of Fe-S cofactors. These processes are particularly important for enzymes requiring [4Fe-4S] clusters, including components of respiratory complex I and lipoic acid synthesis pathways.
Disruption of BOLA3 function can impair mitochondrial energy metabolism and the activity of multiple Fe-S-dependent enzymes. Pathogenic variants in BOLA3 are associated with multiple mitochondrial dysfunctions syndrome type 2 (MMDS2), an autosomal recessive disorder characterized by severe metabolic abnormalities and neurological involvement. Affected individuals may exhibit lactic acidosis, impaired oxidative phosphorylation, and deficiencies involving mitochondrial protein lipoylation. These findings demonstrate the importance of BOLA3 in maintaining the activity of essential mitochondrial metabolic pathways.
BOLA3 is also relevant to investigations of mitochondrial protein homeostasis and the molecular coordination of Fe-S cluster biogenesis. Its functional relationship with NFU1, GLRX5, and other mitochondrial assembly factors illustrates how specialized protein networks support the maturation of respiratory and metabolic enzymes. Studying these interactions can help clarify the consequences of defective cofactor transfer and the relationship between mitochondrial dysfunction and cellular energy metabolism.
NSJ Bioreagents offers this BOLA3 Antibody for research examining mitochondrial Fe-S cluster maturation, respiratory chain function, and inherited disorders of mitochondrial metabolism. Understanding BOLA3-associated protein interactions provides insight into the mechanisms connecting cofactor assembly with mitochondrial enzyme activity and cellular metabolic stability.
Explore our Metabolism Antibodies for additional research tools targeting proteins involved in mitochondrial energy production, iron-sulfur cluster assembly, and cellular metabolic regulation.
Optimal dilution of the BOLA3 Antibody PCRP-BOLA3-1A5 / BolA-like Protein 3 Monoclonal Antibody should be determined by the researcher.
A portion of amino acids 1-103 from the human BOLA3 protein was used as the immunogen for the BOLA3 antibody.
Aliquot the BOLA3 antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.
BolA-like protein 3 antibody, BolA family member 3 antibody, Mitochondrial BolA-like protein 3 antibody, BOLA3 mitochondrial protein antibody
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