Human SDHA Recombinant Protein (His tag)

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SKU:
RPES5594
Product Type:
Recombinant Protein
Species:
Human

Description

Product Name:Human SDHA Recombinant Protein (His tag)
Product Code:RPES5594
Size:20µg
Species:Human
Expression Host:E.coli
Synonyms:Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial, SDHA, Flavoprotein subunit of complex II (Fp), SDHF, SDH2
Mol Mass:68.31 kDa
AP Mol Mass:70 kDa
Tag:N-His & C-His
Purity:> 95 % as determined by reducing SDS-PAGE.
Endotoxin Level:Please contact us for more information.
Bio Activity:Testing in progress
Sequence:Ala 43-Tyr 664
Accession:P31040
Storage: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.
Shipping:This product is provided as lyophilized powder which is shipped with ice packs.
Formulation:Lyophilized from sterile PBS, pH 7.4. Normally 5 % - 8 % trehalose, mannitol and 0.01% Tween80 are added as protectants before lyophilization. Please refer to the specific buffer information in the printed manual.
Reconstitution:Please refer to the printed manual for detailed information.
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.
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