Mouse Anti-BOLA3 Recombinant Antibody (0165) (CBMAB-0165-YC)

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Datasheet

Summary

Host Animal

Mouse

Specificity

Human

Clone

0165

Antibody Isotype

IgG1

Application

IP, FC

Basic Information

Immunogen

Recombinant fragment (around aa1-103) of human BOLA3 protein.

Host Species

Mouse

Specificity

Human

Antibody Isotype

IgG1

Clonality

Monoclonal

Application Notes

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

Application	Note
IP	1-2 µg per 100-500 µg of total protein (1 ml of cell lysate)
FC	1-2 µg/10^6 cells

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

Format

Liquid

Buffer

PBS, 0.05% BSA

Preservative

0.05% sodium azide

Concentration

0.2 mg/ml

Storage

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

Target

Full Name

BolA Family Member 3

Introduction

BOLA3 (BolA family member 3) is a protein that plays an essential role in the production of iron-sulfur (Fe-S) clusters for the normal maturation of lipoate-containing 2-oxoacid dehydrogenases, and for the assembly of the mitochondrial respiratory chain complexes. Disease associated with the gene is multiple mitochondrial dysfunctions syndrome-2.

Entrez Gene ID

388962

UniProt ID

Q53S33

Alternative Names

MMDS2

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.

More Infomation

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)
Proteogenomics
Other Protocols

Associated Products

Related Products

Mouse Anti-BOLA3 Recombinant Antibody (1D12)

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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SDS
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