Gene
aco2
- ID
- ZDB-GENE-030131-1390
- Name
- aconitase 2, mitochondrial
- Symbol
- aco2 Nomenclature History
- Previous Names
- Type
- protein_coding_gene
- Location
- Chr: 12 Mapping Details/Browsers
- Description
- Predicted to enable 4 iron, 4 sulfur cluster binding activity and aconitate hydratase activity. Predicted to be involved in tricarboxylic acid cycle. Predicted to be active in cytosol and mitochondrion. Is expressed in several structures, including adaxial cell; heart tube; musculature system; nervous system; and pronephric duct. Human ortholog(s) of this gene implicated in infantile cerebellar-retinal degeneration and optic atrophy 9. Orthologous to human ACO2 (aconitase 2).
- Genome Resources
- Note
- None
- Comparative Information
-
- All Expression Data
- 6 figures from 2 publications
- Cross-Species Comparison
- High Throughput Data
- Thisse Expression Data
-
- cb1017 (11 images)
Wild Type Expression Summary
- All Phenotype Data
- No data available
- Cross-Species Comparison
- Alliance
Phenotype Summary
Mutations
Human Disease
| Disease Ontology Term | Multi-Species Data | OMIM Term | OMIM Phenotype ID |
|---|---|---|---|
| infantile cerebellar-retinal degeneration | Alliance | Infantile cerebellar-retinal degeneration | 614559 |
| optic atrophy 9 | Alliance | Optic atrophy 9 | 616289 |
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Domain, Family, and Site Summary
| Type | InterPro ID | Name |
|---|---|---|
| Binding_site | IPR018136 | Aconitase family, 4Fe-4S cluster binding site |
| Domain | IPR000573 | Aconitase A/isopropylmalate dehydratase small subunit, swivel domain |
| Domain | IPR001030 | Aconitase/3-isopropylmalate dehydratase large subunit, alpha/beta/alpha domain |
| Family | IPR006248 | Aconitase, mitochondrial-like |
| Family | IPR050926 | Aconitase/IPM Isomerase |
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Domain Details Per Protein
| Protein | Length | Aconitase/3-isopropylmalate dehydratase large subunit, alpha/beta/alpha domain | Aconitase/3-isopropylmalate dehydratase large subunit, alpha/beta/alpha, subdomain 1/3 | Aconitase/3-isopropylmalate dehydratase, swivel | Aconitase A/isopropylmalate dehydratase small subunit, swivel domain | Aconitase, domain 2 | Aconitase family, 4Fe-4S cluster binding site | Aconitase/IPM Isomerase | Aconitase, iron-sulfur domain | Aconitase, mitochondrial-like |
|---|---|---|---|---|---|---|---|---|---|---|
|
UniProtKB:Q6PEI6
|
782 |
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Interactions and Pathways
No data available
Plasmids
No data available
No data available
| Relationship | Marker Type | Marker | Accession Numbers | Citations |
|---|---|---|---|---|
| Contained in | BAC | CH73-139E5 | ZFIN Curated Data | |
| Contained in | BAC | CH211-141O9 | ZFIN Curated Data | |
| Encodes | EST | cb1017 | Thisse et al., 2001 | |
| Encodes | EST | fa10e03 | ||
| Encodes | EST | fb69g04 | ||
| Encodes | EST | fc20c11 | ||
| Encodes | cDNA | MGC:66073 | ZFIN Curated Data |
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| Type | Accession # | Sequence | Length (nt/aa) | Analysis |
|---|---|---|---|---|
| RNA | RefSeq:NM_198908 (1) | 3491 nt | ||
| Genomic | GenBank:BX000473 (1) | 180366 nt | ||
| Polypeptide | UniProtKB:Q6PEI6 (1) | 782 aa |
- LaCoursiere, C.M., Ullmann, J.F.P., Koh, H.Y., Turner, L., Baker, C.M., Robens, B., Shao, W., Rotenberg, A., McGraw, C.M., Poduri, A.H. (2024) Zebrafish models of candidate human epilepsy-associated genes provide evidence of hyperexcitability. iScience. 27:110172110172
- Xu, S.S., Li, Y., Wang, H.P., Chen, W.B., Wang, Y.Q., Song, Z.W., Liu, H., Zhong, S., Sun, Y.H. (2023) Depletion of stearoyl-CoA desaturase (scd) leads to fatty liver disease and defective mating behavior in zebrafish. Zoological research. 44:63-77
- Uszczynska-Ratajczak, B., Sugunan, S., Kwiatkowska, M., Migdal, M., Carbonell-Sala, S., Sokol, A., Winata, C.L., Chacinska, A. (2022) Profiling subcellular localization of nuclear-encoded mitochondrial gene products in zebrafish. Life science alliance. 6(1):
- Yan, Y., Xu, Y., Yang, X., Li, Z., Niu, K., Liu, C., Zhao, M., Xiao, Q., Wu, W. (2022) Electron Transfer Flavoprotein (ETF) α Controls Blood Vessel Development by Regulating Endothelial Mitochondrial Bioenergetics and Oxygen Consumption. Oxidative medicine and cellular longevity. 2022:7969916
- Zhang, Q., He, X., Yao, S., Lin, T., Zhang, L., Chen, D., Chen, C., Yang, Q., Li, F., Zhu, Y.M., Guan, M.X. (2021) Ablation of Mto1 in zebrafish exhibited hypertrophic cardiomyopathy manifested by mitochondrion RNA maturation deficiency. Nucleic acids research. 49(8):4689-4704
- Yang, M.J., Xu, D., Yang, D.X., Li, L., Peng, X.X., Chen, Z.G., Li, H. (2020) Malate enhances survival of zebrafish against Vibrio alginolyticus infection in the same manner as taurine. Virulence. 11:349-364
- Li, Y., Agrawal, I., Gong, Z. (2019) Reversion of tumor hepatocytes to normal hepatocytes during liver tumor regression in an oncogene transgenic zebrafish model. Disease models & mechanisms. 12(10):
- Bayés, À., Collins, M.O., Reig-Viader, R., Gou, G., Goulding, D., Izquierdo, A., Choudhary, J.S., Emes, R.D., Grant, S.G. (2017) Evolution of complexity in the zebrafish synapse proteome. Nature communications. 8:14613
- Chen, W.C., Wang, Z., Missinato, M.A., Park, D.W., Long, D.W., Liu, H.J., Zeng, X., Yates, N.A., Kim, K., Wang, Y. (2016) Decellularized zebrafish cardiac extracellular matrix induces mammalian heart regeneration. Science advances. 2:e1600844
- Motorykin, I., Traber, M.G., Tanguay, R.L., and Maier, C.S. (2014) Proteome-Driven Elucidation of Adaptive Responses to Combined Vitamin E and C Deficiency in Zebrafish. Journal of Proteome Research. 13(3):1647-56
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