This anti-TSFM antibody is a Rabbit Polyclonal antibody detecting TSFM in WB and IHC (p). Suitable for Human, Mouse, Rat, Cow, Dog, Zebrafish (Danio rerio) and Xenopus laevis.
Optimal working dilution should be determined by the investigator.
Restrictions
For Research Use only
Reconstitution
Add 50 μL of distilled water to a final concentration of 1 mg/mL.
Handling Advice
Avoid repeated freezing and thawing.
Storage
4 °C/-20 °C
Storage Comment
Store lyophilized at 2-8 °C or at -20 °C long term. After reconstitution store the antibody undiluted at 2-8 °C for up to one month or in aliquots at -20 °C long term.
The TSFM gene encodes a protein that is expressed in all tissues, with the highest levels of expression in skeletal muscle, liver, and kidney. It associates with the EF-Tu.GDP complex and induces the exchange of GDP to GTP. It remains bound to the aminoacyl-tRNA.EF-Tu.GTP complex up to the GTP hydrolysis stage on the ribosome. Defects in TSFM are the cause of combined oxidative phosphorylation deficiency type 3 (COXPD3). Defects in the mitochondrial oxidative phosphorylation system result in devastating, mainly multisystem, diseases. COXPD3 symptoms include severe metabolic acidosis with encephalomyopathy or with hypertrophic cardiomyopathy. Patients show a severe defect in mitochondrial translation leading to a failure to assemble adequate amounts of three of the oxidative phosphorylation complexesSynonyms: EF-Ts, EF-TsMt, Elongation factor Ts mitochondrial