Mouse Anti-MTFMT (AA 175-389) Recombinant Antibody (CBFYM-2736) (CBMAB-M2929-FY)

This product is mouse antibody that recognizes MTFMT. The antibody CBFYM-2736 can be used for immunoassay techniques such as: WB.
See all MTFMT antibodies

Datasheet

Summary

Host Animal

Mouse

Specificity

Human

Clone

CBFYM-2736

Antibody Isotype

IgG1

Application

Basic Information

Specificity

Human

Antibody Isotype

IgG1

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

Buffer

PBS, pH 7.3, 1% BSA, 50% glycerol

Preservative

0.02% Sodium azide

Concentration

0.46 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.

Epitope

AA 175-389

Target

Full Name

MITOCHONDRIAL METHIONYL-TRNA FORMYLTRANSFERASE

Introduction

The protein encoded by this nuclear gene localizes to the mitochondrion, where it catalyzes the formylation of methionyl-tRNA.

Entrez Gene ID

123263

UniProt ID

Q96DP5

Alternative Names

Mitochondrial Methionyl-TRNA Formyltransferase; FMT1; Methionyl-TRNA Formyltransferase, Mitochondrial; EC 2.1.2.9; COXPD15; MtFMT; FMT

Function

Methionyl-tRNA formyltransferase that formylates methionyl-tRNA in mitochondria and is crucial for translation initiation.

Biological Process

Conversion of methionyl-tRNA to N-formyl-methionyl-tRNA Source: UniProtKB

Cellular Location

Mitochondrion

Involvement in disease

Combined oxidative phosphorylation deficiency 15 (COXPD15):
An autosomal recessive, mitochondrial, neurologic disorder characterized by features of Leigh syndrome and combined oxidative phosphorylation deficiency. Clinical features include mild global developmental delay, white matter abnormalities, ataxia, incoordination, speech and reading difficulties, T2-weighted hyperintensities in the basal ganglia, corpus callosum, and brainstem.
Mitochondrial complex I deficiency, nuclear type 27 (MC1DN27):
A form of mitochondrial complex I deficiency, the most common biochemical signature of mitochondrial disorders, a group of highly heterogeneous conditions characterized by defective oxidative phosphorylation, which collectively affects 1 in 5-10000 live births. Clinical disorders have variable severity, ranging from lethal neonatal disease to adult-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease. MC1DN27 transmission pattern is consistent with autosomal recessive inheritance.

References

Set, K. K., & De Dios, K. (2023). Nonprogressive Mobile Dystonia in MTFMT‐Related Mitochondrial Disease. Movement Disorders Clinical Practice, 10(1), 145.

Howard, C., Dev‐Borman, A., Stokes, J., O'Rourke, D., Gillespie, C., Twomey, E., ... & Boruah, R. (2023). Autonomic instability, arrhythmia and visual impairment in a new presentation of MTFMT‐related mitochondrial disease. JIMD reports, 64(2), 150-155.

Seo, J. H., Hwang, C. S., & Yoo, J. Y. (2020). MTFMT deficiency correlates with reduced mitochondrial integrity and enhanced host susceptibility to intracellular infection. Scientific Reports, 10(1), 11183.

Bennett, J., Kerr, M., Greenway, S. C., Friederich, M. W., Van Hove, J. L., Hittel, D., & Khan, A. (2020). Improved lactate control with dichloroacetate in a case with severe neonatal lactic acidosis due to MTFMT mitochondrial translation disorder. Molecular Genetics and Metabolism Reports, 24, 100616.

Hayhurst, H., de Coo, I. F., Piekutowska‐Abramczuk, D., Alston, C. L., Sharma, S., Thompson, K., ... & Ng, Y. S. (2019). Leigh syndrome caused by mutations in MTFMT is associated with a better prognosis. Annals of clinical and translational neurology, 6(3), 515-524.

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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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