ACO2 Protein (His-GST)
Our Local Distributor
北京 101111
Quick Overview for ACO2 Protein (His-GST) (ABIN7320070)
抗原
See all ACO2 蛋白蛋白类型
宿主
资源
纯度
-
-
标记
- This ACO2 protein is labelled with His-GST.
-
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.
-
过滤
- 0.2 μm filtered
-
内毒素水平
- < 1.0 EU per μg of the protein as determined by the LAL method.
-
Biological Activity Comment
- Not validated for activity
-
-
Aconitase 2, Mitochondrial (ACO2) (AA 1-780) protein (GST tag)
ACO2 宿主: 人 宿主: 小麦胚 Recombinant ELISA, WB, AP, AA
Aconitase 2, Mitochondrial (ACO2) (AA 1-179) protein (GST tag)ACO2 宿主: 人 宿主: 小麦胚 Recombinant ELISA, WB, AP, AA
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
-
-
限制
- 仅限研究用
-
-
-
状态
- Lyophilized
-
缓冲液
-
Lyophilized from sterile 50 mM Tris, 100 mM NaCl, 10 % glycerol, 0.5 mM GSH, pH 8.0
Normally 5 % - 8 % trehalose, mannitol and 0.01 % Tween 80 are added as protectants before lyophilization. -
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.
-
有效期
- 12 months
-
-
- ACO2 (Aconitase 2, Mitochondrial (ACO2))
-
别名
- ACO2/Aconitase 2
-
背景
- Aco-2,Aco3,D10Wsu183e,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.
-
分子量
-
Calculated MW: 110 kDa
Observed MW: 100 kDa
-
UniProt
- Q99KI0
抗原
-
