ACO2 Protein (GST tag,His tag)
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- 抗原 See all ACO2 蛋白
- ACO2 (Aconitase 2, Mitochondrial (ACO2))
- 蛋白类型
- Recombinant
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宿主
- 小鼠
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资源
- Baculovirus infected Insect Cells
- 标记
- This ACO2 protein is labelled with GST tag,His tag.
- 原理
- Recombinant Mouse ACO2/Aconitase 2 Protein (His & GST Tag)
- 序列
- Gln 28-Gln 780
- 产品特性
- 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.
- 纯度
- > 90 % as determined by SDS-PAGE
- 内毒素水平
- < 1.0 EU per μg of the protein as determined by the LAL method.
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- Discover our top product ACO2 蛋白
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Aconitase 2, Mitochondrial (ACO2) (AA 1-780) protein (GST tag)
ACO2 宿主: 人 宿主: 小麦胚 Recombinant ELISA, AP, AA, WB
Aconitase 2, Mitochondrial (ACO2) (AA 1-179) protein (GST tag)ACO2 宿主: 人 宿主: 小麦胚 Recombinant ELISA, AP, AA, WB
Aconitase 2, Mitochondrial (ACO2) protein (Myc-DYKDDDDK Tag)ACO2 宿主: 人 宿主: HEK-293 Cells Recombinant > 80 % as determined by SDS-PAGE and Coomassie blue staining AbP, STD
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- 限制
- 仅限研究用
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- 状态
- Lyophilized
- 溶解方式
- Please refer to the printed manual for detailed information.
- 缓冲液
- Lyophilized from sterile 50 mM Tris, 100 mM NaCl, 10 % glycerol, 0.5 mM GSH, pH 8.0
- 储存条件
- 4 °C,-20 °C,-80 °C
- 储存方法
- 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.
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- 抗原
- ACO2 (Aconitase 2, Mitochondrial (ACO2))
- 别名
- ACO2/Aconitase 2 (ACO2 产品)
- 别名
- DDBDRAFT_0206187 Protein, DDBDRAFT_0230168 Protein, DDB_0206187 Protein, DDB_0230168 Protein, aconitase 2 Protein, F10M23.310 Protein, F10M23_310 Protein, ACONM Protein, ICRD Protein, Aco-2 Protein, Aco3 Protein, D10Wsu183e Protein, cb1017 Protein, wu:fa10e03 Protein, wu:fb69g04 Protein, wu:fc20c11 Protein, aconitase 2 Protein, aconitase 2 S homeolog Protein, aconitate hydratase, mitochondrial Protein, aconitase, mitochondrial Protein, mitochondrial aconitate hydratase Protein, aconitase 2, mitochondrial Protein, Probable aconitate hydratase, mitochondrial Protein, ACO2 Protein, aco2.S Protein, Bm1_07420 Protein, aco2 Protein, ach1 Protein, Aco2 Protein, aco-2 Protein
- 背景
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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.
Synonym: Aco-2,Aco3,D10Wsu183e
- 分子量
- 110 kDa
- UniProt
- Q99KI0
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