Succinate dehydrogenase assembly factor 2 mitochondrial; Succinate dehydrogenase assembly factor 2; mitochondrial; C11orf79; PGL2; SDH5

For Research Use Only. Not for use in diagnostic procedures.

E Coli

> 90% by SDS-PAGE

Liquid. In 20mM Tris-HCl Buffer (pH 8.0) containing 0.2M NaCl, 30% glycerol, 1mM DTT

0.5 mg/ml (determined by Bradford assay) (lot specific)

Can be stored at 4 degree C short term (1-2 weeks).
For long term storage, aliquot and store at -20 degree C or -70 degree C.
Avoid repeated freezing and thawing cycles.

Small volumes of SDHAF2 recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

SDHAF2 is a mitochondrial protein needed for the flavination of a succinate dehydrogenase complex subunit required for activity of the complex. Defects in SDHAF2 are the cause of hereditary paragangliomas type 2 (PGL2), also known as familial non-chromaffin paragangliomas type 2. Paragangliomas refer to rare and mostly benign tumors that arise from any component of the neuroendocrine system. Recombinant human SDHAF2 protein, fused to His-tag at N-terminus, was expressed in E Coli and purified by using conventional chromatography techniques.

Metabolism

SDS-PAGE

19.0kDa (160aa), confirmed by MALDI-TOF

succinate dehydrogenase complex assembly factor 2

succinate dehydrogenase assembly factor 2, mitochondrial

Succinate dehydrogenase assembly factor 2, mitochondrial

SDH assembly factor 2; SDHAF2??[Similar Products]

This gene encodes a mitochondrial protein needed for the flavination of a succinate dehydrogenase complex subunit required for activity of the complex. Mutations in this gene are associated with paraganglioma.[provided by RefSeq, Jul 2010]

Plays an essential role in the assembly of succinate dehydrogenase (SDH), an enzyme complex (also referred to as respiratory complex II) that is a component of both the tricarboxylic acid (TCA) cycle and the mitochondrial electron transport chain, and which couples the oxidation of succinate to fumarate with the reduction of ubiquinone (coenzyme Q) to ubiquinol. Required for flavinylation (covalent attachment of FAD) of the flavoprotein subunit SDHA of the SDH catalytic dimer.

Kunst,H.P., et al. (2011) Clin. Cancer Res. 17 (2), 247-254; Starker,L.F., et al. (2010) Endocrine 38 (3), 397-401

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