Aconitase 2 Cell Lysate | ACO2 cell lysate
Human Aconitase 2 Baculovirus-Insect cells Overexpression Lysate
Gene Names
ACO2; ICRD; ACONM
Applications
Western Blot
Synonyms
Aconitase 2; Human Aconitase 2 Baculovirus-Insect cells Overexpression Lysate; Human ACO2/Aconitase 2 Insect Cell Lysate (WB positive control); aconitase 2; mitochondrial; Human ACONM Overexpression Lysate; Human ICRD Overexpression Lysate; ACO2 cell lysate
Host
Baculovirus-Insect cells
Form/Format
1x Sample Buffer (1x modified RIPA buffer+1x SDS loading buffer).
Modified RIPA Lysis Buffer: 50 mM Tris-HCl pH 7.4, 150 mM NaCl, 1mM EDTA, 1% Triton X-100, 0.1% SDS, 1% Sodium deoxycholate, 1mM PMSF.
Modified RIPA Lysis Buffer: 50 mM Tris-HCl pH 7.4, 150 mM NaCl, 1mM EDTA, 1% Triton X-100, 0.1% SDS, 1% Sodium deoxycholate, 1mM PMSF.
Sequence Positions
990aa
Sequence
Gln28-Gln780
Applicable Applications for ACO2 cell lysate
Western Blot (WB)
Application Notes
WB: Use at an assay dependent dilution.
Species
Human
Recommend Usage
1. Centrifuge the tube for a few seconds and ensure the pellet at the bottom of the tube.2. Re-dissolve the pellet using 200uL pure water and boil for 2-5 min.
Sequence 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.
Preparation Method
Cell lysate was prepared by homogenization of the over-expressed cells in ice-cold modified RIPA Lysis Buffer with cocktail of protease inhibitors. Cell debris was removed by centrifugation. Protein concentration was determined by Bradford assay (protein assay, Microplate Standard assay). The cell lysate was boiled for 5 min in 1 x SDS loading buffer (50mM Tris-HCl pH 6.8, 12.5% glycerol, 1% sodium dodecylsulfate, 0.01% bromophenol blue) containing 5% b-mercaptoethanol, and lyophilized.
Preparation and Storage
Store at 4 degree C for up to twelve months from date of receipt. After re-dissolution, aliquot and store at -80 degree C for up to twelve months. Avoid repeated freeze-thaw cycles.
Samples are stable for up to twelve months from date of receipt.
Samples are stable for up to twelve months from date of receipt.
Related Product Information for ACO2 cell lysate
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.
This Human Aconitase 2 overexpression lysate was created in Baculovirus-Insect cells and intented for use as a Western blot (WB) positive control. Purification of Aconitase 2 protein from the overexpression lysate was verified.
This Human Aconitase 2 overexpression lysate was created in Baculovirus-Insect cells and intented for use as a Western blot (WB) positive control. Purification of Aconitase 2 protein from the overexpression lysate was verified.
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 #
NCBI GeneID
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
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
Research Articles on ACO2
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Product Notes
The ACO2 aco2 (Catalog #AAA8114257) is a Cell Lysate produced from Baculovirus-Insect cells and is intended for research purposes only. The product is available for immediate purchase. The immunogen sequence is 990aa. AAA Biotech's Aconitase 2 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). WB: Use at an assay dependent dilution. Researchers should empirically determine the suitability of the ACO2 aco2 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. The amino acid sequence is listed below: Gln28-Gln7 80. It is sometimes possible for the material contained within the vial of "Aconitase 2, Cell Lysate" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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