Mouse anti-Human MICU1 Monoclonal Antibody

Uniprot Id

Q9BPX6

Target Species

Human

Target Name

MICU1

Target Full Name

Calcium uptake protein 1, mitochondrial

Target Function

Key regulator of mitochondrial calcium uniporter (MCU) that senses calcium level via its EF-hand domains. 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. Acts as a gatekeeper of MCU at low concentration of calcium, preventing channel opening. Enhances MCU opening at high calcium concentration, allowing a rapid response of mitochondria to calcium signals generated in the cytoplasm. Regulates glucose-dependent insulin secretion in pancreatic beta-cells by regulating mitochondrial calcium uptake. Induces T-helper 1-mediated autoreactivity, which is accompanied by the release of IFNG.

Target Involvement

Myopathy with extrapyramidal signs (MPXPS)

Target Subcellular Location

Mitochondrion inner membrane; Single-pass membrane protein. Mitochondrion intermembrane space.

Target Protein Families

MICU1 family, MICU1 subfamily

Target Tissue Specificity

Expressed in epithelial cell lines. Strongly expressed in epidermal keratinocytes and dermal endothelial cells.

Target Synonyms

Allergen Hom s 4; ara CALC; atopy related autoantigen ; atopy related autoantigen CALC ; Atopy-related autoantigen CALC; CALC; calcium binding atopy related autoantigen 1; Calcium uptake protein 1; calcium uptake protein 1, mitochondrial ; Calcium-binding atopy-related autoantigen 1; CBARA1; EFHA3; FLJ12684; MICU1; MICU1_HUMAN; mitochondrial; mitochondrial calcium uptake 1 ; MPXPS

Target Background

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.