Optimal working dilution should be determined by the investigator.
限制
仅限研究用
溶解方式
Add 50 μL of distilled water to a final concentration of 1 mg/mL.
注意事项
Avoid repeated freezing and thawing.
储存条件
4 °C/-20 °C
储存方法
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