Contents

ACO2 — Aconitase 2

🧬 Gene Info

Gene Symbol	ACO2
Full Name	Aconitase 2
Chromosome	22q13.2
Protein Type	Enzyme
Function	is a mitochondrial enzyme that catalyzes the stereospecific isomerization of citrate to isocitrate via cis-aconitate in the second step of the tricarboxylic acid (TCA) cycle[@beinert1996].
Primary Expression	all nucleated cells:
Amino Acids	778 aa
Exons	18
Ensembl ID	ENSG00000100412
GeneCards	ACO2
Human Protein Atlas	ACO2
Four functional domains	Organized around the 4Fe-4S cluster
4Fe-4S cluster binding site	Essential for catalytic activity and redox sensing
Active site	Contains three conserved cysteine residues coordinating the iron-sulfur cluster
Citrate → cis-Aconitate → Isocitrate	Stereochemical isomerization
Associated Diseases	neurodegeneration
Interactions	ATP, ATP5A1, CYCS, GLUD1, HSPA9, IMMUNE INFILTRATION
KG Connections	81 knowledge graph edges
Databases	GeneCards UniProt NCBI Gene HPA STRING

Knowledge Graph