ACO2

ACO2
Identifiers
Aliases	ACO2, ACONM, ICRD, OCA8, HEL-S-284, OPA9, aconitase 2
External IDs	MGI: 87880 HomoloGene: 856 GeneCards: ACO2
Gene ontology Molecular function • iron ion binding • 4 iron, 4 sulfur cluster binding • iron-sulfur cluster binding • 3 iron, 4 sulfur cluster binding • metal ion binding • lyase activity • aconitate hydratase activity Cellular component • myelin sheath • mitochondrial matrix • mitochondrion Biological process • citrate metabolic process • metabolic process • tricarboxylic acid cycle • generation of precursor metabolites and energy • isocitrate metabolic process • liver development • response to isolation stress Sources:Amigo / QuickGO
Orthologs
Species	Human	Mouse
Entrez	50	11429
Ensembl	ENSG00000100412	ENSMUSG00000022477
UniProt	Q99798	Q99KI0
RefSeq (mRNA)	NM_001098	NM_080633
RefSeq (protein)	NP_001089	NP_542364
Location (UCSC)	Chr 22: 41.47 – 41.53 Mb	Chr 15: 81.87 – 81.92 Mb
PubMed search

50

11429

ENSG00000100412

ENSMUSG00000022477

Q99798

Q99KI0

NM_001098

NM_080633

NP_001089

NP_542364

Aconitase 2, mitochondrial is a protein that in humans is encoded by the ACO2 gene.

The secondary structure of ACO2 consists of numerous alternating alpha helices and beta sheets (SCOP classification: α/β alternating). The tertiary structure reveals that the active site is buried in the middle of the enzyme, and, since there is only one subunit, there is no quaternary structure. Aconitase consists of four domains: three of the domains are tightly compact, and the fourth domain is more flexible, allowing for conformational changes. The ACO2 protein contains a 4Fe-4S iron-sulfur cluster. This iron sulfur cluster does not have the typical function of participating in oxidation-reduction reactions, but rather facilitates the elimination of the citrate hydroxyl group by holding the group in a certain conformation and orientation. It is at this 4Fe-4S site that citrate or isocitrate binds to initiate catalysis. The rest of the active site is made up of the following residues: Gln72, Asp100, His101, Asp165, Ser166, His167, His147, Glu262, Asn258, Cys358, Cys421, Cys424, Cys358, Cys421, Asn446, Arg447, Arg452, Asp568, Ser642, Ser643, Arg644, Arg580. Their functions have yet to be elucidated.

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 . 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.

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