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Function
Calcium uptake protein 1 or Mitochondrial calcium uptake protein (MICU1) is essential in preventing mitochondrial Ca+2 overload[1]. For more details see Calcium uptake protein 1.
Mitochondrial Calcium uptake protein 2 (MICU2) behaves as an inhibitor of mitochondrial Ca+2 uniporter at low cystolic Ca+2[2].
Mitochondrial Calcium uptake protein 3 (MICU3), an EF-hand containing protein, behaves as an enhancer of mitochondrial Ca+2 uniporter-dependent mitochondrial Ca+2 uptake[3].
Disease
Mutations in MICU1 cause a brain and muscle disorder.
Structural highlights
MICU1 contains 2 Ca+2 binding sites. A canonical EF1 site and a canonical EF4 site[4].
3D Structures of calcium uptake protein
Calcium uptake protein 3D structures
- ↑ Logan CV, Szabadkai G, Sharpe JA, Parry DA, Torelli S, Childs AM, Kriek M, Phadke R, Johnson CA, Roberts NY, Bonthron DT, Pysden KA, Whyte T, Munteanu I, Foley AR, Wheway G, Szymanska K, Natarajan S, Abdelhamed ZA, Morgan JE, Roper H, Santen GW, Niks EH, van der Pol WL, Lindhout D, Raffaello A, De Stefani D, den Dunnen JT, Sun Y, Ginjaar I, Sewry CA, Hurles M, Rizzuto R, Duchen MR, Muntoni F, Sheridan E. Loss-of-function mutations in MICU1 cause a brain and muscle disorder linked to primary alterations in mitochondrial calcium signaling. Nat Genet. 2014 Feb;46(2):188-93. doi: 10.1038/ng.2851. Epub 2013 Dec 15. PMID:24336167 doi:https://dx.doi.org/10.1038/ng.2851
- ↑ Matesanz-Isabel J, Arias-del-Val J, Alvarez-Illera P, Fonteriz RI, Montero M, Alvarez J. Functional roles of MICU1 and MICU2 in mitochondrial Ca(2+) uptake. Biochim Biophys Acta. 2016 Jun;1858(6):1110-7. doi: 10.1016/j.bbamem.2016.02.022., Epub 2016 Feb 18. PMID:26903221 doi:https://dx.doi.org/10.1016/j.bbamem.2016.02.022
- ↑ Patron M, Granatiero V, Espino J, Rizzuto R, De Stefani D. MICU3 is a tissue-specific enhancer of mitochondrial calcium uptake. Cell Death Differ. 2019 Jan;26(1):179-195. doi: 10.1038/s41418-018-0113-8. Epub, 2018 May 3. PMID:29725115 doi:https://dx.doi.org/10.1038/s41418-018-0113-8
- ↑ Wang L, Yang X, Li S, Wang Z, Liu Y, Feng J, Zhu Y, Shen Y. Structural and mechanistic insights into MICU1 regulation of mitochondrial calcium uptake. EMBO J. 2014 Feb 10. PMID:24514027 doi:https://dx.doi.org/10.1002/embj.201386523
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