Comparison

Recombinant Mouse ACO2/Aconitase 2 Protein (His & GST Tag)

Item no. ELS-PKSM040388-100ug
Manufacturer Elabscience
Amount 100 ug
Quantity options 100 ug 20 ug
Category
Type Proteins
Format Lyophilized Powder
Specific against Mouse (Murine, Mus musculus)
Host Insect Cells, Baculovirus-Infected Insect Cells
Conjugate/Tag HIS, GST
Purity > 90 % as determined by reducing SDS-PAGE.
Sequence Gln 28-Gln 780
ECLASS 10.1 32160409
ECLASS 11.0 32160409
UNSPSC 12352202
Alias Aco-2, Aco3, D10Wsu183e
Similar products Aco-2, Aco3, D10Wsu183e
Shipping Condition Cool pack
Available
Manufacturer - Category
Recombinant Proteins / Others
Manufacturer - Conjugate / Tag
N-His-GST
Shipping Temperature
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℃. Reconstituted protein solution can be stored at 4-8℃ for 2-7 days. Aliquots of reconstituted samples are stable at < -20℃ for 3 months.
Calculated Molecular Weight
110 kDa
Observed Molecular Weight
100 kDa
Background
A homozygous missense mutation was identified in the ACO2 gene (c.124T>G p.Phe414Val) that segregated with HSP complicated by intellectual disability and microcephaly. Lymphoblastoid cell lines of homozygous carrier patients revealed significantly decreased activity of the mitochondrial aconitase enzyme and defective mitochondrial respiration. ACO2 encodes mitochondrial aconitase, an essential enzyme in the Krebs cycle. Recessive mutations in this gene have been previously associated with cerebellar ataxia. We found homozygous or compound heterozygous missense and frameshift mutations in the gene encoding mitochondrial aconitase (ACO2), a tricarboxylic acid cycle enzyme, catalysing interconversion of citrate into isocitrate. Unlike wild type ACO2, all mutant ACO2 proteins failed to complement the respiratory growth of a yeast aco1-deletion strain. The study shows that autosomal recessive ACO2 mutations can cause either isolated or syndromic optic neuropathy. This observation identifies ACO2 as the second gene responsible for non-syndromic autosomal recessive optic neuropathies and provides evidence for a genetic overlap between isolated and syndromic forms, giving further support to the view that optic atrophy is a hallmark of defective mitochondrial energy supply.
Endotoxin
< 1.0 EU per μg of the protein as determined by the LAL method.
Reconstitution
Please refer to the printed manual for detailed information.
Formulation
Lyophilized from sterile 50mM Tris, 100mM NaCl, 10% glycerol, 0.5mM GSH, pH 8.0
Normally 5% - 8% trehalose, mannitol and 0.01% Tween 80 are added as protectants before lyophilization.
Please refer to the specific buffer information in the printed manual.
Activity
Not validated for activity
Protein Construction
A DNA sequence encoding the mouse ACO2 (Q99KI0) (Gln 28-Gln 780) was fused with the N-terminal polyhistidine-tagged GST tag at the N-terminus.

Note: The presented information and documents (Manual, Product Datasheet, Safety Datasheet and Certificate of Analysis) correspond to our latest update and should serve for orientational purpose only. We do not guarantee the topicality. We would kindly ask you to make a request for specific requirements, if necessary.

All products are intended for research use only (RUO). Not for human, veterinary or therapeutic use.

Amount: 100 ug
Available: In stock
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