Gene
mcu
- ID
- ZDB-GENE-061013-24
- Name
- mitochondrial calcium uniporter
- Symbol
- mcu Nomenclature History
- Previous Names
-
- ccdc109a
- wu:fi24a01
- zgc:153607 (1)
- Type
- protein_coding_gene
- Location
- Chr: 13 Mapping Details/Browsers
- Description
- Enables identical protein binding activity. Acts upstream of or within several processes, including calcium import into the mitochondrion; convergent extension involved in gastrulation; and mitochondrial calcium ion homeostasis. Located in mitochondrion. Is expressed in brain; head; liver; pericardial region; and visceral fat. Used to study cardiomyopathy. Orthologous to human MCU (mitochondrial calcium uniporter).
- Genome Resources
- Note
- None
- Comparative Information
-
- All Expression Data
- 9 figures from 8 publications
- Cross-Species Comparison
- High Throughput Data
- Thisse Expression Data
- No data available
Wild Type Expression Summary
- All Phenotype Data
- 11 figures from 6 publications
- Cross-Species Comparison
- Alliance
Phenotype Summary
Mutations
| Allele | Type | Localization | Consequence | Mutagen | Supplier |
|---|---|---|---|---|---|
| ct149aGt | Transgenic insertion | Unknown | Unknown | DNA | |
| la2434 | Allele with one deletion | Exon 1 | Frameshift | TALEN | |
| la027461Tg | Transgenic insertion | Unknown | Unknown | DNA | |
| mn111 | Allele with one deletion | Unknown | Frameshift | TALEN | |
| mn0599Gt | Transgenic insertion | Unknown | Frameshift | DNA | |
| sa22206 | Allele with one point mutation | Unknown | Premature Stop | ENU | |
| sh214 | Allele with one delins | Unknown | Unknown | CRISPR | |
| tpl124 | Allele with one deletion | Unknown | Unknown | CRISPR | |
| w249a | Allele with one delins | Exon 5 | Premature Stop | CRISPR |
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| Targeting Reagent | Created Alleles | Citations |
|---|---|---|
| CRISPR1-mcu | Tomar et al., 2019 | |
| CRISPR2-mcu | Tomar et al., 2019 | |
| CRISPR3-mcu | Tomar et al., 2019 | |
| CRISPR4-mcu | Soman et al., 2019 | |
| MO1-mcu | N/A | Prudent et al., 2013 |
| MO2-mcu | N/A | (2) |
| MO3-mcu | N/A | Soman et al., 2017 |
| TALEN1-mcu | (2) | |
| TALEN2-mcu | Langenbacher et al., 2020 |
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Human Disease
| Human Disease | Fish | Conditions | Citations |
|---|---|---|---|
| cardiomyopathy | mcula2434/la2434 | standard conditions | Langenbacher et al., 2020 |
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Domain, Family, and Site Summary
Domain Details Per Protein
| Protein | Additional Resources | Length | Calcium uniporter protein, C-terminal | MCU family |
|---|---|---|---|---|
| UniProtKB:K0A5D6 | InterPro | 376 | ||
| UniProtKB:Q08BI9 | InterPro | 376 |
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Interactions and Pathways
No data available
Plasmids
| Relationship | Marker Type | Marker | Accession Numbers | Citations |
|---|---|---|---|---|
| Contained in | BAC | CH73-57A19 | ZFIN Curated Data | |
| Contained in | Fosmid | CH1073-92F7 | ZFIN Curated Data | |
| Contained in | Fosmid | CH1073-423J5 | ZFIN Curated Data | |
| Contained in | Fosmid | ZFOS-289A11 | ZFIN Curated Data | |
| Encodes | EST | fi24a01 | ||
| Encodes | cDNA | MGC:153607 | ZFIN Curated Data |
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| Type | Accession # | Sequence | Length (nt/aa) | Analysis |
|---|---|---|---|---|
| RNA | RefSeq:NM_001077325 (1) | 1898 nt | ||
| Genomic | GenBank:CU856520 (2) | 108204 nt | ||
| Polypeptide | UniProtKB:K0A5D6 (1) | 376 aa |
- Grepper, D., Tabasso, C., Zanou, N., Aguettaz, A.K.F., Castro-Sepulveda, M., Ziegler, D.V., Lagarrigue, S., Arribat, Y., Martinotti, A., Ebrahimi, A., Daraspe, J., Fajas, L., Amati, F. (2024) BCL2L13 at endoplasmic reticulum-mitochondria contact sites regulates calcium homeostasis to maintain skeletal muscle function. iScience. 27:110510110510
- Tanwar, J., Ahuja, K., Sharma, A., Sehgal, P., Ranjan, G., Sultan, F., Agrawal, A., D'Angelo, D., Priya, A., Yenamandra, V.K., Singh, A., Raffaello, A., Madesh, M., Rizzuto, R., Sivasubbu, S., Motiani, R.K. (2024) Mitochondrial calcium uptake orchestrates vertebrate pigmentation via transcriptional regulation of keratin filaments. PLoS Biology. 22:e3002895e3002895
- Xu, Y., Zhang, S., Bao, Y., Luan, J., Fu, Z., Sun, M., Zhao, X., Feng, X. (2024) Melatonin protects zebrafish pancreatic development and physiological rhythms from sodium propionate-induced disturbances via the hypothalamic-pituitary-thyroid axis. Journal of the science of food and agriculture. 104(12):7454-7463
- Sabharwal, A., Campbell, J.M., Schwab, T.L., WareJoncas, Z., Wishman, M.D., Ata, H., Liu, W., Ichino, N., Hunter, D.E., Bergren, J.D., Urban, M.D., Urban, R.M., Holmberg, S.R., Kar, B., Cook, A., Ding, Y., Xu, X., Clark, K.J., Ekker, S.C. (2022) A Primer Genetic Toolkit for Exploring Mitochondrial Biology and Disease Using Zebrafish. Genes. 13(8):
- Cleghorn, W.M., Burrell, A.L., Giarmarco, M.M., Brock, D.C., Wang, Y., Chambers, Z.S., Du, J., Kollman, J.M., Brockerhoff, S.E. (2021) A highly conserved zebrafish IMPDH retinal isoform produces the majority of guanine and forms dynamic protein filaments in photoreceptor cells. The Journal of biological chemistry. 298(1):101441
- Wiweger, M., Majewski, L., Adamek-Urbanska, D., Wasilewska, I., Kuznicki, J. (2021) npc2-Deficient Zebrafish Reproduce Neurological and Inflammatory Symptoms of Niemann-Pick Type C Disease. Frontiers in Cellular Neuroscience. 15:647860
- Bisbach, C.M., Hutto, R.A., Poria, D., Cleghorn, W.M., Abbas, F., Vinberg, F., Kefalov, V.J., Hurley, J.B., Brockerhoff, S.E. (2020) Mitochondrial Calcium Uniporter (MCU) deficiency reveals an alternate path for Ca2+ uptake in photoreceptor mitochondria. Scientific Reports. 10:16041
- Langenbacher, A.D., Shimizu, H., Hsu, W., Zhao, Y., Borges, A., Koehler, C., Chen, J.N. (2020) Mitochondrial Calcium Uniporter Deficiency in Zebrafish Causes Cardiomyopathy With Arrhythmia. Frontiers in Physiology. 11:617492
- Collins, M.M., Ahlberg, G., Hansen, C.V., Guenther, S., Marín-Juez, R., Sokol, A.M., El-Sammak, H., Piesker, J., Hellsten, Y., Olesen, M.S., Stainier, D.Y.R., Lundegaard, P.R. (2019) Early sarcomere and metabolic defects in a zebrafish pitx2c cardiac arrhythmia model. Proceedings of the National Academy of Sciences of the United States of America. 116(48):24115-24121
- Hutto, R.A., Bisbach, C.M., Abbas, F., Brock, D.C., Cleghorn, W.M., Parker, E.D., Bauer, B.H., Ge, W., Vinberg, F., Hurley, J.B., Brockerhoff, S.E. (2019) Increasing Ca2+ in photoreceptor mitochondria alters metabolites, accelerates photoresponse recovery, and reveals adaptations to mitochondrial stress. Cell death and differentiation. 27(3):1067-1085
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