Alternative Name(s):

Flavoprotein subunit of complex II ;Fp

Species: (Organism)

Homo sapiens (Human)

Gene Names:

SDHA

Tag info:

N-terminal GST-tagged

Target Protein AA Sequence:

SAKVSDSISAQYPVVDHEFDAVVVGAGGAGLRAAFGLSEAGFNTACVTKLFPTRSHTVAAQGGINAALGNMEEDNWRWHFYDTVKGSDWLGDQDAIHYMTEQAPAAVVELENYGMPFSRTEDGKIYQRAFGGQSLKFGKGGQAHRCCCVADRTGHSLLHTLYGRSLRYDTSYFVEYFALDLLMENGECRGVIALCIEDGSIHRIRAKNTVVATGGYGRTYFSCTSAHTSTGDGTAMITRAGLPCQDLEFV

Expression Region:

44-293aa

Subcellular Location:

Mitochondrion inner membrane, Peripheral membrane protein, Matrix side

Tissue Specificity:

Protein Length:

Partial

Pathway:

OxidativePhosphorylation

Mol. Weight:

54.1 kDa

Purity:

Greater than 90% as determined by SDS-PAGE.

Form:

Liquid or Lyophilized powder

Buffer:

If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol. If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.

Research Areas:

Transport

Function:

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 (coenzyme Q)

Involvement in disease:

Mitochondrial complex II deficiency (MT-C2D); Leigh syndrome (LS); Cardiomyopathy, dilated 1GG (CMD1GG); Paragangliomas 5 (PGL5)

Relevance:

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 (coenzyme Q). Can act as a tumor suppressor.

Reconstitution:

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.

Protein Families:

FAD-dependent oxidoreductase 2 family, FRD/SDH subfamily

Reference:

Compound heterozygous mutations in the flavoprotein gene of the respiratory chain complex II in a patient with Leigh syndrome.Parfait B., Chretien D., Roetig A., Marsac C., Munnich A., Rustin P.Hum. Genet. 106:236-243(2000)