Mouse Anti-FDX2 Recombinant Antibody (CBXF-1736) (CBMAB-F3271-CQ)

This product is a mouse antibody that recognizes FDX2. The antibody CBXF-1736 can be used for immunoassay techniques such as: WB, IP, IF, ELISA.
See all FDX2 antibodies

Datasheet

Summary

Host Animal

Mouse

Specificity

Human

Clone

CBXF-1736

Antibody Isotype

IgG

Application

WB, IP, IF, ELISA

Basic Information

Specificity

Human

Antibody Isotype

IgG

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!]

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

Ferredoxin 2

Introduction

This gene encodes a member of the ferredoxin family. The encoded protein contains a 2Fe-2S ferredoxin-type domain and is essential for heme A and Fe/S protein biosynthesis. Mutation in FDX1L gene is associated with mitochondrial muscle myopathy.

Entrez Gene ID

112812

UniProt ID

Q6P4F2

Alternative Names

FDX1L

Research Area

Essential for heme A and Fe/S protein biosynthesis.

Cellular Location

Mitochondrion; Mitochondrion matrix

Involvement in disease

Mitochondrial myopathy, episodic, with optic atrophy and reversible leukoencephalopathy (MEOAL):
An autosomal recessive neuromuscular disorder characterized by childhood onset of recurrent episodes of proximal weakness and myalgia often precipitated by exercise, infections or low temperature. Additional features are optic atrophy, axonal polyneuropathy, and reversible or partially reversible leukoencephalopathy.

References

Schulz, V., Basu, S., Freibert, S. A., Webert, H., Boss, L., Mühlenhoff, U., ... & Lill, R. (2022). Functional spectrum and specificity of mitochondrial ferredoxins FDX1 and FDX2. Nature Chemical Biology, 1-12.

Aggarwal, A., Pillai, N. R., Billington Jr, C. J., Schema, L., & Berry, S. A. (2022). Rare presentation of FDX2‐related disorder and untargeted global metabolomics findings. American Journal of Medical Genetics Part A, 188(4), 1239-1244.

Weiler, B. D., Brück, M. C., Kothe, I., Bill, E., Lill, R., & Mühlenhoff, U. (2020). Mitochondrial [4Fe-4S] protein assembly involves reductive [2Fe-2S] cluster fusion on ISCA1–ISCA2 by electron flow from ferredoxin FDX2. Proceedings of the National Academy of Sciences, 117(34), 20555-20565.

Gervason, S., Larkem, D., Mansour, A. B., Botzanowski, T., Müller, C. S., Pecqueur, L., ... & D’autréaux, B. (2019). Physiologically relevant reconstitution of iron-sulfur cluster biosynthesis uncovers persulfide-processing functions of ferredoxin-2 and frataxin. Nature communications, 10(1), 1-12.

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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

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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