Mouse Anti-BOLA3 Recombinant Antibody (1D12) (CBMAB-A0848-LY)

The product is antibody recognizes BOLA3. The antibody 1D12 immunoassay techniques such as: WB, ELISA.
See all BOLA3 antibodies

Datasheet

Summary

Host Animal

Mouse

Specificity

Human

Clone

1D12

Antibody Isotype

IgG2a, κ

Application

WB, ELISA

Basic Information

Immunogen

BOLA3 (AAH42036.1, 1 a.a. ~ 68 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

Specificity

Human

Antibody Isotype

IgG2a, κ

Clonality

Monoclonal

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format

Liquid

Purity

> 95% Purity determined by SDS-PAGE.

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name

BolA Family Member 3

Entrez Gene ID

388962

UniProt ID

Q53S33

Function

Acts as a mitochondrial iron-sulfur (Fe-S) cluster assembly factor that facilitates (Fe-S) cluster insertion into a subset of mitochondrial proteins. Probably acts together with NFU1 (PubMed:27532772).

Cellular Location

Mitochondrion

Involvement in disease

Multiple mitochondrial dysfunctions syndrome 2 with hyperglycinemia (MMDS2): A severe disorder of systemic energy metabolism, resulting in weakness, respiratory failure, lack of neurologic development, lactic acidosis, hyperglycinemia and early death. Some patients show failure to thrive, pulmonary hypertension, hypotonia and irritability. Biochemical features include severe combined deficiency of the 2-oxoacid dehydrogenases, defective lipoic acid synthesis and reduction in activity of mitochondrial respiratory chain complexes.

References

Zhu, M., & Xiao, S. (2021). Expression profiles and prognostic values of BolA family members in ovarian cancer. Journal of Ovarian Research, 14(1), 1-12.

Bai, N., Ma, J., Alimujiang, M., Xu, J., Hu, F., Xu, Y., ... & Yang, Y. (2021). Bola3 Regulates Beige Adipocyte Thermogenesis via Maintaining Mitochondrial Homeostasis and Lipolysis. Frontiers in Endocrinology, 11, 1004.

Yu, Q., Tai, Y. Y., Tang, Y., Zhao, J., Negi, V., Culley, M. K., ... & Chan, S. Y. (2019). BOLA (BolA Family Member 3) deficiency controls endothelial metabolism and glycine homeostasis in pulmonary hypertension. Circulation, 139(19), 2238-2255.

Yu, Q., Tai, Y. Y., Tang, Y., Zhao, J., Bertero, T., Negi, V., ... & Chan, S. Y. (2018). Bola3 Deficiency Controls Endothelial Metabolism and Glycine Homeostasis in Pulmonary Hypertension. Circulation, 138(Suppl_1), A11422-A11422.

Nasta, V., Giachetti, A., Ciofi-Baffoni, S., & Banci, L. (2017). Structural insights into the molecular function of human [2Fe-2S] BOLA1-GRX5 and [2Fe-2S] BOLA3-GRX5 complexes. Biochimica et Biophysica Acta (BBA)-General Subjects, 1861(8), 2119-2131.

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Protocols

Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

Associated Products

Related Products

Mouse Anti-BOLA3 Antibody (1A5)

Isotype control

For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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