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

Basic Information
Formulations & Storage [For reference only, actual COA shall prevail!]
Target
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).
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
Mitochondrion inner membrane
Mitochondrion intermembrane space
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.
Mitochondrial intermembrane: 53-476
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Please try the standard protocols which include: protocols, troubleshooting and guide.
Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols
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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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