Aconitase 2 Recombinant Protein | ACO2 recombinant protein

Aconitase 2 Protein, Human, Recombinant (His & GST Tag)

Cat. Num. AAA8120601

• Gene Names: ACO2; ICRD; ACONM
• Purity: >85% as determined by SDS-PAGE
• Synonyms: Aconitase 2; Aconitase 2 Protein; Human; Recombinant (His & GST Tag); Human ACO2/Aconitase 2 Protein (His & GST Tag); ACONM Protein; ICRD Protein; aconitase 2; mitochondrial; ACO2 recombinant protein

• Host: Baculovirus-Insect Cells
• Purity/Purification: >85% as determined by SDS-PAGE
• Form/Format: Lyophilized from sterile 20mM Tris, 500mM NaCl, 10% glycerol, pH8.0, 0.3mM DTT. Normally 5%-8% trehalose, mannitol and 0.01% Tween80 are added as protectants before lyophilization. Please refer to the specific buffer information in the hard copy of CoA.
• Sequence: Gln28-Gln780

• Species: Human
• Endotoxin: <1.0EU per ug of the protein as determined by the LAL method
• Predicted N Terminal: Met
• Tag: N-GST & His
• Protein Construction: A DNA sequence encoding the human ACO2 (Q99798)(Gln 28-Gln 780) was fused with the N-terminal polyhistidine-tagged GST tag at the N-terminus.
• Reconstitution: A hardcopy of COA with reconstitution instruction is sent along with the products. Please refer to it for detailed information.
• Preparation and Storage: Samples are stable for up to twelve months from date of receipt at -20 degree C to -80 degree C. Store it under sterile conditions at -20 degree C to -80 degree C. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.; In general, recombinant proteins are provided as lyophilized powder which are shipped at ambient temperature. Bulk packages of recombinant proteins are provided as frozen liquid.; They are shipped out with blue ice.

SDS-Page

Related Product Information for ACO2 recombinant protein

Background: A homozygous missense mutation was identified in the ACO2 gene (c.1240T>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.

References

Robbins AH, et al. (1989) The structure of aconitase. Proteins. 5 (4): 289-312.Lauble H, et al. (1992) Crystal structures of aconitase with isocitrate and nitroisocitrate bound. Biochemistry. 31 (10): 2735-48.Robbins AH, et al. (1989) Structure of activated aconitase: formation of the 4Fe-4S cluster in the crystal. Proc Natl Acad Sci. 86 (10): 3639-43.

NCBI and Uniprot Product Information

• NCBI GI #: 6686275
• NCBI GeneID: 50
• UniProt Accession #: Q99798; O75809; Q5JZ41; Q6FHX0; Q8TAQ6
• Molecular Weight: 780
• NCBI Official Full Name: Aconitate hydratase, mitochondrial
• NCBI Official Synonym Full Names: aconitase 2, mitochondrial
• NCBI Official Symbol: ACO2
• NCBI Official Synonym Symbols: ICRD; ACONM
• NCBI Protein Information: aconitate hydratase, mitochondrial; citrate hydro-lyase
• UniProt Protein Name: Aconitate hydratase, mitochondrial
• Protein Family: Aconitate hydratase
• UniProt Gene Name: ACO2
• UniProt Synonym Gene Names: Aconitase
• UniProt Entry Name: ACON_HUMAN

NCBI Description

The protein encoded by this gene belongs to the aconitase/IPM isomerase family. It is an enzyme that catalyzes the interconversion of citrate to isocitrate via cis-aconitate in the second step of the TCA cycle. This protein is encoded in the nucleus and functions in the mitochondrion. It was found to be one of the mitochondrial matrix proteins that are preferentially degraded by the serine protease 15(PRSS15), also known as Lon protease, after oxidative modification. [provided by RefSeq, Jul 2008]

Uniprot Description

ACO2: Catalyzes the isomerization of citrate to isocitrate via cis-aconitate. Monomer. Belongs to the aconitase/IPM isomerase family.

Protein type: Lyase; EC 4.2.1.3; Carbohydrate Metabolism - glyoxylate and dicarboxylate; Carbohydrate Metabolism - citrate (TCA) cycle; Mitochondrial

Chromosomal Location of Human Ortholog: 22q13.2

Cellular Component: mitochondrion; mitochondrial matrix; nucleus

Molecular Function: iron ion binding; 4 iron, 4 sulfur cluster binding; aconitate hydratase activity; 3 iron, 4 sulfur cluster binding

Biological Process: isocitrate metabolic process; cellular metabolic process; generation of precursor metabolites and energy; tricarboxylic acid cycle; citrate metabolic process

Disease: Infantile Cerebellar-retinal Degeneration; Optic Atrophy 8