Recombinant Mouse Aconitase 2 Protein (His & GST Tag)
Beta LifeScience
SKU/CAT #: BLPSN-0059
Recombinant Mouse Aconitase 2 Protein (His & GST Tag)
Beta LifeScience
SKU/CAT #: BLPSN-0059
Collections: Other recombinant proteins, Recombinant proteins
Our products are highly customizable to meet your specific needs. You can choose options such as endotoxin removal, liquid or lyophilized forms, preferred tags, and the desired functional sequence range for proteins. Submitting a written inquiry expedites the quoting process.
Product Overview
Tag | His&GST |
Host Species | Mouse |
Accession | Q99KI0 |
Synonym | Aco-2, Aco3, D10Wsu183e |
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. |
Description | A DNA sequence encoding the mouse ACO2 (Q99KI0) (Gln 28-Gln 780) was fused with the N-terminal His-tagged GST tag at the N-terminus. |
Source | Baculovirus-Insect Cells |
Predicted N Terminal | Met |
AA Sequence | Gln 28-Gln 780 |
Molecular Weight | The secreted recombinant mouse ACO2/GST chimera consists of 990 a.a. and has a calculated molecular mass of 110 kDa. The recombinant protein migrates as an approximately 100 kDa band in SDS-PAGE under reducing conditions. |
Purity | >90% as determined by SDS-PAGE |
Endotoxin | < 1.0 EU per μg of the protein as determined by the LAL method |
Bioactivity | Please contact us for detailed information |
Formulation | Lyophilized from sterile 50mM Tris, 100mM NaCl, 10% gly, 0.5mM GSH, pH 8.0. |
Stability | The recombinant proteins are stable for up to 1 year from date of receipt at -70°C. |
Usage | For Research Use Only |
Storage | Store the protein under sterile conditions at -20°C to -80°C. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles. |