Recombinant Human SDHA protein (His tag)

Specifications

Catalog number

PDEH100987-01

Size / count

20 µg

Dry ice

No

Hazardous

No

Discontinued

No

Background

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.

Abbreviation

SDHA

Synonyms

SDHA; Flavoprotein subunit of complex II (Fp) ; SDH2; SDHF; Succinate dehydrogenase [ubiquinone] flavoprotein subunit; mitochondrial

UniProt

P31040

Accession Number

P31040

Expression System

E.coli

Tag

N-His & C-His

Sequence

Ala 43-Tyr 664

Field of Research

Cancer; Cell Biology; Metabolism; signal Transduction; Tag and Cell Markers

Endotoxin

< 10 EU/mg of the protein as determined by the LAL method.

Purity

> 95% as determined by reducing SDS-PAGE.

Bioactivity

Not validated for activity

Reconstitution

It is recommended that sterile water be added to the vial to prepare a stock solution of 0.5 mg/mL. Concentration is measured by UV-Vis.

Shipping Conditions

This product is provided as lyophilized powder which is shipped with ice packs.

Storage Conditions

Generally, lyophilized proteins are stable for up to 12 months when stored at -20 to -80°C. Reconstituted protein solution can be stored at 4-8°C for 2-7 days. Aliquots of reconstituted samples are stable at < -20°C for 3 months.

Calculated Molecular Weight

68.3 kDa

Observed Molecular Weight

70 kDa

Species

Human