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Mouse Anti-MICU1 Recombinant Antibody (CBFYM-2217) (CBMAB-M2400-FY)

This product is mouse antibody that recognizes MICU1. The antibody CBFYM-2217 can be used for immunoassay techniques such as: IHC, WB.
See all MICU1 antibodies

Summary

Host Animal
Mouse
Specificity
Human, Mouse, Rat
Clone
CBFYM-2217
Application
IHC, WB

Basic Information

Immunogen
Recombinant Protein
Specificity
Human, Mouse, Rat
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS, pH 7.2, 50% glycerol
Preservative
0.02% Sodium azide
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Mitochondrial Calcium Uptake 1
Introduction
This gene encodes an essential regulator of mitochondrial Ca2+ uptake under basal conditions. The encoded protein interacts with the mitochondrial calcium uniporter, a mitochondrial inner membrane Ca2+ channel, and is essential in preventing mitochondrial Ca2+ overload, which can cause excessive production of reactive oxygen species and cell stress. Alternatively spliced transcript variants encoding different isoforms have been described.
Entrez Gene ID
Human10367
Mouse216001
Rat365567
UniProt ID
HumanQ9BPX6
MouseQ8VCX5
RatQ6P6Q9
Alternative Names
Mitochondrial Calcium Uptake 1; Calcium-Binding Atopy-Related Autoantigen 1; Atopy-Related Autoantigen CALC; Ara CALC; CBARA1; CALC
Function
Key regulator of mitochondrial calcium uniporter (MCU) that senses calcium level via its EF-hand domains (PubMed:20693986, PubMed:23101630, PubMed:23747253, PubMed:24313810, PubMed:24332854, PubMed:24503055, PubMed:24560927, PubMed:26341627, PubMed:26903221, PubMed:27099988).

MICU1 and MICU2 form a disulfide-linked heterodimer that stimulates and inhibits MCU activity, depending on the concentration of calcium. MICU1 acts both as an activator or inhibitor of mitochondrial calcium uptake (PubMed:26903221).

Acts as a gatekeeper of MCU at low concentration of calcium, preventing channel opening (PubMed:26903221).

Enhances MCU opening at high calcium concentration, allowing a rapid response of mitochondria to calcium signals generated in the cytoplasm (PubMed:24560927, PubMed:26903221).

Regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake (PubMed:22904319).

Induces T-helper 1-mediated autoreactivity, which is accompanied by the release of IFNG (PubMed:16002733).
Biological Process
Calcium import into the mitochondrion Source: ComplexPortal
Calcium ion import Source: UniProtKB
Defense response Source: ProtInc
Mitochondrial calcium ion homeostasis Source: ComplexPortal
Mitochondrial calcium ion transmembrane transport Source: UniProtKB
Positive regulation of mitochondrial calcium ion concentration Source: UniProtKB
Protein homooligomerization Source: UniProtKB
Regulation of cellular hyperosmotic salinity response Source: UniProtKB
Cellular Location
Mitochondrion
Mitochondrion inner membrane
Mitochondrion intermembrane space
Involvement in disease
Myopathy with extrapyramidal signs (MPXPS):
An autosomal recessive disorder characterized by early-onset proximal muscle weakness with a static course and moderately to grossly elevated serum creatine kinase levels accompanied by learning difficulties. Most patients develop subtle extrapyramidal motor signs that progress to a debilitating disorder of involuntary movement with variable features, including chorea, tremor, dystonic posturing and orofacial dyskinesia. Additional variable features include ataxia, microcephaly, ophthalmoplegia, ptosis, optic atrophy and axonal peripheral neuropathy.
An homozygous partial MICU1 deletion is responsible for a disorder manifesting in childhood with fatigue, lethargy and muscle weakness. The disease is caused by variants affecting the gene represented in this entry.
Topology
Helical: 34-52
Mitochondrial intermembrane: 53-476

Tomar, D., Thomas, M., Garbincius, J. F., Kolmetzky, D. W., Salik, O., Jadiya, P., ... & Elrod, J. W. (2023). MICU1 regulates mitochondrial cristae structure and function independently of the mitochondrial Ca2+ uniporter channel. Science Signaling, 16(782), eabi8948.

Tsai, C. W., Liu, T. Y., Chao, F. Y., Tu, Y. C., Rodriguez, M. X., Van Keuren, A. M., ... & Tsai, M. F. (2023). Evidence supporting the MICU1 occlusion mechanism and against the potentiation model in the mitochondrial calcium uniporter complex. Proceedings of the National Academy of Sciences, 120(16), e2217665120.

Rodríguez-Prados, M., Berezhnaya, E., Castromonte, M. T., Menezes-Filho, S. L., Paillard, M., & Hajnóczky, G. (2023). MICU1 occludes the mitochondrial calcium uniporter in divalent-free conditions. Proceedings of the National Academy of Sciences, 120(19), e2218999120.

Rodríguez-Prados, M., Huang, K. T., Márta, K., Paillard, M., Csordás, G., Joseph, S. K., & Hajnóczky, G. (2023). MICU1 controls the sensitivity of the mitochondrial Ca2+ uniporter to activators and inhibitors. Cell Chemical Biology, 30(6), 606-617.

Kohlschmidt, N., Elbracht, M., Czech, A., Häusler, M., Phan, V., Töpf, A., ... & Roos, A. (2021). Molecular pathophysiology of human MICU1 deficiency. Neuropathology and applied neurobiology, 47(6), 840-855.

Di Marco, G., Vallese, F., Jourde, B., Bergsdorf, C., Sturlese, M., De Mario, A., ... & Mammucari, C. (2020). A high-throughput screening identifies MICU1 targeting compounds. Cell reports, 30(7), 2321-2331.

Wu, W., Shen, Q., Zhang, R., Qiu, Z., Wang, Y., Zheng, J., & Jia, Z. (2020). The structure of the MICU 1‐MICU 2 complex unveils the regulation of the mitochondrial calcium uniporter. The EMBO Journal, 39(19), e104285.

Wu, W., Shen, Q., Lei, Z., Qiu, Z., Li, D., Pei, H., ... & Jia, Z. (2019). The crystal structure of MICU 2 provides insight into Ca2+ binding and MICU 1‐MICU 2 heterodimer formation. EMBO reports, 20(9), e47488.

Gottschalk, B., Klec, C., Leitinger, G., Bernhart, E., Rost, R., Bischof, H., ... & Graier, W. F. (2019). MICU1 controls cristae junction and spatially anchors mitochondrial Ca2+ uniporter complex. Nature communications, 10(1), 3732.

Marchi, S., Corricelli, M., Branchini, A., Vitto, V. A. M., Missiroli, S., Morciano, G., ... & Pinton, P. (2019). Akt‐mediated phosphorylation of MICU 1 regulates mitochondrial Ca2+ levels and tumor growth. The EMBO journal, 38(2), e99435.

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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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