Recombinant Human Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial (SDHA), partial | CSB-EP020903HU1

Recombinant Human Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial (SDHA), partial | CSB-EP020903HU1 | Cusabio

Alternative Name(s): Flavoprotein subunit of complex II ;Fp

Gene Names: SDHA

Research Areas: Transport

Organism: Homo sapiens (Human)

AA Sequence: SAKVSDSISAQYPVVDHEFDAVVVGAGGAGLRAAFGLSEAGFNTACVTKLFPTRSHTVAAQGGINAALGNMEEDNWRWHFYDTVKGSDWLGDQDAIHYMTEQAPAAVVELENYGMPFSRTEDGKIYQRAFGGQSLKFGKGGQAHRCCCVADRTGHSLLHTLYGRSLRYDTSYFVEYFALDLLMENGECRGVIALCIEDGSIHRIRAKNTVVATGGYGRTYFSCTSAHTSTGDGTAMITRAGLPCQDLEFV

Source: E.coli

Tag Info: N-terminal GST-tagged

Expression Region: 44-293aa

Sequence Info: Partial

MW: 54.1 kDa

Purity: Greater than 90% as determined by SDS-PAGE.

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.

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)

Storage: The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself. Generally, the shelf life of liquid form is 6 months at -20?/-80?. The shelf life of lyophilized form is 12 months at -20?/-80?.

Notes: Repeated freezing and thawing is not recommended. Store working aliquots at 4? for up to one week.

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)

Subcellular Location: Mitochondrion inner membrane, Peripheral membrane protein, Matrix side

Protein Families: FAD-dependent oxidoreductase 2 family, FRD/SDH subfamily

Tissue Specificity:

Paythway: OxidativePhosphorylation

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.

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.

Uniprot ID: P31040

HGNC Database Link: HGNC

UniGene Database Link: UniGene

KEGG Database Link: KEGG

STRING Database Link: STRING

OMIM Database Link: OMIM