Flavoprotein (FP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone.Defects in SDHA are a cause of mitochondrial complex II deficiency (MT-C2D). A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations. Clinical features include psychomotor regression in infants, poor growth with lack of speech development, severe spastic quadriplegia, dystonia, progressive leukoencephalopathy, muscle weakness, exercise intolerance, cardiomyopathy. Some patients manifest Leigh syndrome or Kearns-Sayre syndrome.

Dettagli prodotto

• Codice: PDEH100987_20μg
• Marca: Elabscience
• Specie target: Human
• Host: E.coli
• Confezionamento: Lyophilized from sterile PBS, pH 7.4.<br/>Normally 5%-8% trehalose, mannitol and 0.01% Tween 80 are added as protectants before lyophilization.<br/>Please refer to the specific buffer information in the printed manual.
• Link: Apri link
• Stoccaggio: Generally, lyophilized proteins are stable for up to 12 months when stored at -20 to -80℃. Reconstituted protein solution can be stored at 4-8℃ for 2-7 days. Aliquots of reconstituted samples are stable at < -20℃ for 3 months.
• Simbolo target: SDHA
• Ig target: SDHA;Flavoprotein subunit of complex II (Fp);SDH2;SDHF;Succinate dehydrogenase [ubiquinone] flavoprotein subunit;mitochondrial
• Area di ricerca: Cancer;Cell Biology;Metabolism;signal Transduction;Tag and Cell Markers