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Entry version 168 (16 Oct 2019)
Sequence version 1 (01 Jun 2001)
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Protein

Calcium uptake protein 1, mitochondrial

Gene

MICU1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score:

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the ‘protein existence’ evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

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).12 Publications

Caution

Was initially thought to act as an inhibitor of MCU based on effects following MICU1 depletion (PubMed:20693986, PubMed:23101630). However, depletion of MICU1 also eliminates MICU2, explaining the initial conclusion. It was later shown to act as a stimulator of MCU activity instead (PubMed:24560927).3 Publications
The topology is subject to discussion. According to some reports, localizes at the outer surface of the mitochondrion inner membrane (PubMed:20693986, PubMed:24332854). According to another publication, forms an intramembrane hairpin loop without crossing the membrane (PubMed:23747253). Recent studies rather suggest that it contains a transmembrane region that crosses the mitochondrial inner membrane, with the main part of the protein localized in the mitochondrial intermembrane space (PubMed:26387864, PubMed:26489515).5 Publications

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">Function</a> section specifies the position(s) of the calcium-binding region(s) within the protein. One common calcium-binding motif is the EF-hand, but other calcium-binding motifs also exist.<p><a href='/help/ca_bind' target='_top'>More...</a></p>Calcium bindingi231 – 2421PROSITE-ProRule annotation1 PublicationAdd BLAST12
Calcium bindingi421 – 4322PROSITE-ProRule annotation1 PublicationAdd BLAST12

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

GO - Biological processi

<p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

Biological processCalcium transport, Ion transport, Transport
LigandCalcium, Metal-binding

Enzyme and pathway databases

Reactome - a knowledgebase of biological pathways and processes

More...
Reactomei
R-HSA-8949215 Mitochondrial calcium ion transport
R-HSA-8949664 Processing of SMDT1

Protein family/group databases

Transport Classification Database

More...
TCDBi
8.A.44.1.1 the mitochondrial ef hand ca(2+) uniporter regulator (micu) family

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>Protein namesi
Recommended name:
Calcium uptake protein 1, mitochondrial
Alternative name(s):
Atopy-related autoantigen CALC1 Publication
Short name:
ara CALC1 Publication
Calcium-binding atopy-related autoantigen 11 Publication
Allergen: Hom s 41 Publication
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: ‘Name’, ‘Synonyms’, ‘Ordered locus names’ and ‘ORF names’.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
Name:MICU1
Synonyms:CALC1 Publication, CBARA1
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the ‘taxonomic identifier’ or ‘taxid’.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
  • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes_manual">proteome</a> can consist of several components. <br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome 10

Organism-specific databases

Human Gene Nomenclature Database

More...
HGNCi
HGNC:1530 MICU1

Online Mendelian Inheritance in Man (OMIM)

More...
MIMi
605084 gene

neXtProt; the human protein knowledge platform

More...
neXtProti
NX_Q9BPX6

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte & Seán O’Donoghue; Source: COMPARTMENTS

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/subcellular_location_section">'Subcellular location'</a> section describes the extent of a membrane-spanning region of the protein. It denotes the presence of both alpha-helical transmembrane regions and the membrane spanning regions of beta-barrel transmembrane proteins.<p><a href='/help/transmem' target='_top'>More...</a></p>Transmembranei34 – 52HelicalSequence analysisAdd BLAST19
<p>This subsection of the <a href="http://www.uniprot.org/help/subcellular_location_section">'Subcellular location'</a> section describes the subcellular compartment where each non-membrane region of a membrane-spanning protein is found.<p><a href='/help/topo_dom' target='_top'>More...</a></p>Topological domaini53 – 476Mitochondrial intermembrane1 PublicationAdd BLAST424

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

<p>This subsection of the ‘Pathology and Biotech’ section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>Involvement in diseasei

Myopathy with extrapyramidal signs (MPXPS)1 Publication
The disease is caused by mutations affecting the gene represented in this entry. The complex phenotype is due to alterations in mitochondrial calcium signaling characterized by increased mitochondrial Ca(2+) load (PubMed:24336167).1 Publication
Disease descriptionAn 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.
Related information in OMIM
An homozygous partial MICU1 deletion is responsible for a disorder manifesting in childhood with fatigue, lethargy and muscle weakness. The disease is caused by mutations affecting the gene represented in this entry.1 Publication

<p>This subsection of the <a href="http://www.uniprot.org/manual/pathology_and_biotech_section">'Pathology and Biotech'</a> section is used for proteins that cause an allergic reaction in mammals. We usually specify in which species the protein is allergenic.<p><a href='/help/allergenic_properties' target='_top'>More...</a></p>Allergenic propertiesi

Causes an allergic reaction in human. Binds to IgE from atopic dermatitis (AD) patients. Identified as an IgE autoantigen in atopic dermatitis (AD) patients with severe skin manifestations.1 Publication

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/manual/pathology_and_biotech_section">'Pathology and Biotech'</a> section describes the effect of the experimental mutation of one or more amino acid(s) on the biological properties of the protein.<p><a href='/help/mutagen' target='_top'>More...</a></p>Mutagenesisi99 – 102KKKK → EQEQ: Abolishes interaction with EMRE/SMDT1 while maintaining intedraction with MICU2SO. 1 Publication4
Mutagenesisi221R → A: Abolishes homooligomerization. 1 Publication1
Mutagenesisi231D → A: Abolishes mitochondrial Ca(2+) uptake; when associated with A-242; A-421 and A-432. 3 Publications1
Mutagenesisi242E → A or K: Abolishes mitochondrial Ca(2+) uptake; when associated with A-231; A-421 and A-432. 3 Publications1
Mutagenesisi376D → A: Abolishes homooligomerization. 1 Publication1
Mutagenesisi383 – 385FYH → AYA: Abolishes homooligomerization. 1 Publication3
Mutagenesisi421D → A: Abolishes mitochondrial Ca(2+) uptake; when associated with A-231; A-242 and A-432. 3 Publications1
Mutagenesisi432E → A: Abolishes mitochondrial Ca(2+) uptake; when associated with A-231; A-242 and A-421. 3 Publications1
Mutagenesisi432E → K: Loss of function; when associated with A-421. 3 Publications1
Mutagenesisi463C → A: Abolishes interchain disulfide bond and heterodimer formation with MICU2. 1 Publication1

Keywords - Diseasei

Allergen

Organism-specific databases

DisGeNET

More...
DisGeNETi
10367

MalaCards human disease database

More...
MalaCardsi
MICU1
MIMi615673 phenotype

Open Targets

More...
OpenTargetsi
ENSG00000107745

Orphanet; a database dedicated to information on rare diseases and orphan drugs

More...
Orphaneti
401768 Proximal myopathy with extrapyramidal signs

The Pharmacogenetics and Pharmacogenomics Knowledge Base

More...
PharmGKBi
PA26110

Miscellaneous databases

Pharos NIH Druggable Genome Knowledgebase

More...
Pharosi
Q9BPX6

Protein family/group databases

Allergome; a platform for allergen knowledge

More...
Allergomei
3325 Hom s 4.0101
414 Hom s 4

Polymorphism and mutation databases

BioMuta curated single-nucleotide variation and disease association database

More...
BioMutai
MICU1

Domain mapping of disease mutations (DMDM)

More...
DMDMi
74761192

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a transit peptide.<p><a href='/help/transit' target='_top'>More...</a></p>Transit peptidei1 – 33MitochondrionSequence analysis1 PublicationAdd BLAST33
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a polypeptide chain in the mature protein following processing.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_000032299034 – 476Calcium uptake protein 1, mitochondrialAdd BLAST443

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the PTM / Processing":/help/ptm_processing_section section describes the positions of cysteine residues participating in disulfide bonds.<p><a href='/help/disulfid' target='_top'>More...</a></p>Disulfide bondi463Interchain (with C-413 in MICU2)1 Publication

Keywords - PTMi

Disulfide bond

Proteomic databases

Encyclopedia of Proteome Dynamics

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EPDi
Q9BPX6

jPOST - Japan Proteome Standard Repository/Database

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jPOSTi
Q9BPX6

MassIVE - Mass Spectrometry Interactive Virtual Environment

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MassIVEi
Q9BPX6

MaxQB - The MaxQuant DataBase

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MaxQBi
Q9BPX6

PaxDb, a database of protein abundance averages across all three domains of life

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PaxDbi
Q9BPX6

PeptideAtlas

More...
PeptideAtlasi
Q9BPX6

PRoteomics IDEntifications database

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PRIDEi
Q9BPX6

ProteomicsDB: a multi-organism proteome resource

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ProteomicsDBi
78588 [Q9BPX6-1]
78589 [Q9BPX6-2]
78590 [Q9BPX6-3]
78591 [Q9BPX6-4]
78592 [Q9BPX6-5]

PTM databases

iPTMnet integrated resource for PTMs in systems biology context

More...
iPTMneti
Q9BPX6

Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

More...
PhosphoSitePlusi
Q9BPX6

SwissPalm database of S-palmitoylation events

More...
SwissPalmi
Q9BPX6

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

<p>This subsection of the ‘Expression’ section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms. By default, the information is derived from experiments at the mRNA level, unless specified ‘at protein level’. <br></br>Examples: <a href="http://www.uniprot.org/uniprot/P92958#expression">P92958</a>, <a href="http://www.uniprot.org/uniprot/Q8TDN4#expression">Q8TDN4</a>, <a href="http://www.uniprot.org/uniprot/O14734#expression">O14734</a><p><a href='/help/tissue_specificity' target='_top'>More...</a></p>Tissue specificityi

Expressed in epithelial cell lines. Strongly expressed in epidermal keratinocytes and dermal endothelial cells.2 Publications

Gene expression databases

Bgee dataBase for Gene Expression Evolution

More...
Bgeei
ENSG00000107745 Expressed in 228 organ(s), highest expression level in cerebellar hemisphere

ExpressionAtlas, Differential and Baseline Expression

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ExpressionAtlasi
Q9BPX6 baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

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Genevisiblei
Q9BPX6 HS

Organism-specific databases

Human Protein Atlas

More...
HPAi
HPA037479
HPA037480

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

<p>This subsection of the <a href="http://www.uniprot.org/help/interaction_section">'Interaction'</a> section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

Homohexamer; in absence of calcium (PubMed:24514027).

Forms a homohexamer in absence of calcium and rearranges into a heterodimer in presence of calcium (PubMed:26489515, PubMed:24514027). Heterodimer; disulfide-linked; heterodimerizes with MICU2 (PubMed:24560927). The heterodimer formed with MICU2 associates with MCU at low calcium concentration and dissociates from MCU at high calcium level (PubMed:26387864).

Component of the uniplex complex, composed of MCU, MCUB, MICU1, MICU2 and EMRE/SMDT1 (PubMed:24231807).

Interacts (via polybasic region) with EMRE/SMDT1; the interaction is direct (PubMed:27099988).

Interacts (via polybasic region) with MCU (via coiled coil domains); the interaction is direct and precedes formation of the heterodimer with MICU2 (PubMed:21685886, PubMed:23101630, PubMed:23178883, PubMed:24332854, PubMed:26387864).

Interacts with SLC25A23 (PubMed:24430870).

Interacts with CHCHD4/MIA40; which introduces the interchain disulfide bond with MICU2 (PubMed:26387864).

11 Publications

<p>This subsection of the '<a href="http://www.uniprot.org/help/interaction_section%27">Interaction</a> section provides information about binary protein-protein interactions. The data presented in this section are a quality-filtered subset of binary interactions automatically derived from the <a href="http://www.ebi.ac.uk/intact/">IntAct database</a>. It is updated on a monthly basis. Each binary interaction is displayed on a separate line.<p><a href='/help/binary_interactions' target='_top'>More...</a></p>Binary interactionsi

GO - Molecular functioni

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGrid)

More...
BioGridi
115646, 35 interactors

CORUM comprehensive resource of mammalian protein complexes

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CORUMi
Q9BPX6

Database of interacting proteins

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DIPi
DIP-53438N

Protein interaction database and analysis system

More...
IntActi
Q9BPX6, 29 interactors

Molecular INTeraction database

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MINTi
Q9BPX6

STRING: functional protein association networks

More...
STRINGi
9606.ENSP00000354415

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

Secondary structure

1476
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

SWISS-MODEL Repository - a database of annotated 3D protein structure models

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SMRi
Q9BPX6

Database of comparative protein structure models

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ModBasei
Search...

Protein Data Bank in Europe - Knowledge Base

More...
PDBe-KBi
Search...

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/family_and_domains_section">Family and Domains</a> section describes the position and type of a domain, which is defined as a specific combination of secondary structures organized into a characteristic three-dimensional structure or fold.<p><a href='/help/domain' target='_top'>More...</a></p>Domaini218 – 253EF-hand 1PROSITE-ProRule annotationAdd BLAST36
Domaini408 – 443EF-hand 2PROSITE-ProRule annotationAdd BLAST36

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Family and Domains’ section describes a region of interest that cannot be described in other subsections.<p><a href='/help/region' target='_top'>More...</a></p>Regioni99 – 110Polybasic region1 PublicationAdd BLAST12
Regioni455 – 465C-helix region1 PublicationAdd BLAST11

<p>This subsection of the ‘Family and domains’ section provides general information on the biological role of a domain. The term ‘domain’ is intended here in its wide acceptation, it may be a structural domain, a transmembrane region or a functional domain. Several domains are described in this subsection.<p><a href='/help/domain_cc' target='_top'>More...</a></p>Domaini

The C-helix is required for assembling the Ca2+-free homohexamer (PubMed:24514027). It also plays a key role in mitochondrial calcium uptake, probably by mediating interaction with MICU2 (PubMed:24503055, PubMed:24514027).2 Publications
The EF-hand domains have high affinity for calcium and act as sensors of calcium levels (PubMed:23101630, PubMed:24560927).2 Publications

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Belongs to the MICU1 family. MICU1 subfamily.Curated

Keywords - Domaini

Repeat, Transit peptide, Transmembrane, Transmembrane helix

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

More...
eggNOGi
KOG2643 Eukaryota
ENOG410ZSC1 LUCA

Ensembl GeneTree

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GeneTreei
ENSGT00950000183079

InParanoid: Eukaryotic Ortholog Groups

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InParanoidi
Q9BPX6

KEGG Orthology (KO)

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KOi
K22827

Database of Orthologous Groups

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OrthoDBi
707988at2759

Database for complete collections of gene phylogenies

More...
PhylomeDBi
Q9BPX6

TreeFam database of animal gene trees

More...
TreeFami
TF313815

Family and domain databases

Integrated resource of protein families, domains and functional sites

More...
InterProi
View protein in InterPro
IPR011992 EF-hand-dom_pair
IPR018247 EF_Hand_1_Ca_BS
IPR002048 EF_hand_dom
IPR039800 MICU1/2/3

The PANTHER Classification System

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PANTHERi
PTHR12294 PTHR12294, 1 hit

Pfam protein domain database

More...
Pfami
View protein in Pfam
PF13202 EF-hand_5, 1 hit

Simple Modular Architecture Research Tool; a protein domain database

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SMARTi
View protein in SMART
SM00054 EFh, 2 hits

Superfamily database of structural and functional annotation

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SUPFAMi
SSF47473 SSF47473, 2 hits

PROSITE; a protein domain and family database

More...
PROSITEi
View protein in PROSITE
PS00018 EF_HAND_1, 2 hits
PS50222 EF_HAND_2, 2 hits

<p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence_length">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>. The information is filed in different subsections. The current subsections and their content are listed below:<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequences (5+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is in its mature form or if it represents the precursor.<p><a href='/help/sequence_processing' target='_top'>More...</a></p>Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 5 <p>This subsection of the ‘Sequence’ section lists the alternative protein sequences (isoforms) that can be generated from the same gene by a single or by the combination of up to four biological events (alternative promoter usage, alternative splicing, alternative initiation and ribosomal frameshifting). Additionally, this section gives relevant information on each alternative protein isoform.<p><a href='/help/alternative_products' target='_top'>More...</a></p> isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 5 described isoforms and 7 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: Q9BPX6-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the <div> <p><b>What is the canonical sequence?</b><p><a href='/help/canonical_and_isoforms' target='_top'>More...</a></p>canonicali sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide
        10         20         30         40         50
MFRLNSLSAL AELAVGSRWY HGGSQPIQIR RRLMMVAFLG ASAVTASTGL
60 70 80 90 100
LWKRAHAESP PCVDNLKSDI GDKGKNKDEG DVCNHEKKTA DLAPHPEEKK
110 120 130 140 150
KKRSGFRDRK VMEYENRIRA YSTPDKIFRY FATLKVISEP GEAEVFMTPE
160 170 180 190 200
DFVRSITPNE KQPEHLGLDQ YIIKRFDGKK ISQEREKFAD EGSIFYTLGE
210 220 230 240 250
CGLISFSDYI FLTTVLSTPQ RNFEIAFKMF DLNGDGEVDM EEFEQVQSII
260 270 280 290 300
RSQTSMGMRH RDRPTTGNTL KSGLCSALTT YFFGADLKGK LTIKNFLEFQ
310 320 330 340 350
RKLQHDVLKL EFERHDPVDG RITERQFGGM LLAYSGVQSK KLTAMQRQLK
360 370 380 390 400
KHFKEGKGLT FQEVENFFTF LKNINDVDTA LSFYHMAGAS LDKVTMQQVA
410 420 430 440 450
RTVAKVELSD HVCDVVFALF DCDGNGELSN KEFVSIMKQR LMRGLEKPKD
460 470
MGFTRLMQAM WKCAQETAWD FALPKQ
Length:476
Mass (Da):54,351
Last modified:June 1, 2001 - v1
<p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> Checksum:iD83C3A38F4B28FDF
GO
Isoform 2 (identifier: Q9BPX6-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     394-403: VTMQQVARTV → GKGTIFMGRR
     404-476: Missing.

Note: No experimental confirmation available.
Show »
Length:403
Mass (Da):45,975
Checksum:i2E7EB518965C6750
GO
Isoform 3 (identifier: Q9BPX6-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     179-179: K → KTE

Note: No experimental confirmation available.
Show »
Length:478
Mass (Da):54,582
Checksum:i6814001923EC4054
GO
Isoform 4 (identifier: Q9BPX6-4) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-198: Missing.
     199-218: GECGLISFSDYIFLTTVLST → MKRIYTYRRAKEIFKDTPKA

Note: No experimental confirmation available.
Show »
Length:278
Mass (Da):32,122
Checksum:i92C650B8F161E1C2
GO
Isoform 5 (identifier: Q9BPX6-5) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-198: Missing.
     199-218: GECGLISFSDYIFLTTVLST → MVSLKAKLNHLRQSMLKQKA

Note: No experimental confirmation available.
Show »
Length:278
Mass (Da):31,931
Checksum:iE2736D73550EAAED
GO

<p>In eukaryotic reference proteomes, unreviewed entries that are likely to belong to the same gene are computationally mapped, based on gene identifiers from Ensembl, EnsemblGenomes and model organism databases.<p><a href='/help/gene_centric_isoform_mapping' target='_top'>More...</a></p>Computationally mapped potential isoform sequencesi

There are 7 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
F6XEV2F6XEV2_HUMAN
Calcium uptake protein 1, mitochond...
MICU1
478Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
A0A0U1RRK1A0A0U1RRK1_HUMAN
Calcium uptake protein 1, mitochond...
MICU1
480Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
A0A286YF11A0A286YF11_HUMAN
Calcium uptake protein 1, mitochond...
MICU1
482Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
S4R3V1S4R3V1_HUMAN
Calcium uptake protein 1, mitochond...
MICU1
388Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
S4R3D7S4R3D7_HUMAN
Calcium uptake protein 1, mitochond...
MICU1
212Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
E9PQV6E9PQV6_HUMAN
Calcium uptake protein 1, mitochond...
MICU1
246Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
S4R3W3S4R3W3_HUMAN
Calcium uptake protein 1, mitochond...
MICU1
154Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>

<p>This subsection of the ‘Sequence’ section reports difference(s) between the protein sequence shown in the UniProtKB entry and other available protein sequences derived from the same gene.<p><a href='/help/sequence_caution' target='_top'>More...</a></p>Sequence cautioni

The sequence BAB14187 differs from that shown. Reason: Erroneous initiation. Truncated N-terminus.Curated
The sequence CAA76830 differs from that shown. Contaminating sequence. Sequence of unknown origin in the N-terminal part.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section reports difference(s) between the canonical sequence (displayed by default in the entry) and the different sequence submissions merged in the entry. These various submissions may originate from different sequencing projects, different types of experiments, or different biological samples. Sequence conflicts are usually of unknown origin.<p><a href='/help/conflict' target='_top'>More...</a></p>Sequence conflicti54R → G in CAB55915 (PubMed:11230166).Curated1
Sequence conflicti449K → E in BAG60593 (PubMed:14702039).Curated1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section describes the sequence of naturally occurring alternative protein isoform(s). The changes in the amino acid sequence may be due to alternative splicing, alternative promoter usage, alternative initiation, or ribosomal frameshifting.<p><a href='/help/var_seq' target='_top'>More...</a></p>Alternative sequenceiVSP_0398901 – 198Missing in isoform 4 and isoform 5. 1 PublicationAdd BLAST198
Alternative sequenceiVSP_031979179K → KTE in isoform 3. 1 Publication1
Alternative sequenceiVSP_039891199 – 218GECGL…TVLST → MKRIYTYRRAKEIFKDTPKA in isoform 4. 1 PublicationAdd BLAST20
Alternative sequenceiVSP_039892199 – 218GECGL…TVLST → MVSLKAKLNHLRQSMLKQKA in isoform 5. 1 PublicationAdd BLAST20
Alternative sequenceiVSP_031980394 – 403VTMQQVARTV → GKGTIFMGRR in isoform 2. 1 Publication10
Alternative sequenceiVSP_031981404 – 476Missing in isoform 2. 1 PublicationAdd BLAST73

Sequence databases

Select the link destinations:

EMBL nucleotide sequence database

More...
EMBLi

GenBank nucleotide sequence database

More...
GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

More...
DDBJi
Links Updated
AK023318 mRNA Translation: BAG51182.1
AK296086 mRNA Translation: BAG58841.1
AK298347 mRNA Translation: BAG60593.1
AL117423 mRNA Translation: CAB55915.1
AC091769 Genomic DNA No translation available.
AL513185 Genomic DNA No translation available.
AL356009 Genomic DNA No translation available.
CH471083 Genomic DNA Translation: EAW54459.1
BC004190 mRNA Translation: AAH04190.1
BC004216 mRNA Translation: AAH04216.1
BC016641 mRNA Translation: AAH16641.1
AK022697 mRNA Translation: BAB14187.1 Different initiation.
Y17711 mRNA Translation: CAA76830.1 Sequence problems.

The Consensus CDS (CCDS) project

More...
CCDSi
CCDS55714.1 [Q9BPX6-4]
CCDS55715.1 [Q9BPX6-1]

Protein sequence database of the Protein Information Resource

More...
PIRi
T17225

NCBI Reference Sequences

More...
RefSeqi
NP_001182447.1, NM_001195518.1 [Q9BPX6-1]
NP_001182448.1, NM_001195519.1 [Q9BPX6-4]
NP_006068.2, NM_006077.3 [Q9BPX6-3]

Genome annotation databases

Ensembl eukaryotic genome annotation project

More...
Ensembli
ENST00000361114; ENSP00000354415; ENSG00000107745 [Q9BPX6-1]
ENST00000398763; ENSP00000381747; ENSG00000107745 [Q9BPX6-5]
ENST00000418483; ENSP00000402470; ENSG00000107745 [Q9BPX6-4]

Database of genes from NCBI RefSeq genomes

More...
GeneIDi
10367

KEGG: Kyoto Encyclopedia of Genes and Genomes

More...
KEGGi
hsa:10367

UCSC genome browser

More...
UCSCi
uc001jtb.3 human [Q9BPX6-1]

Keywords - Coding sequence diversityi

Alternative splicing

<p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK023318 mRNA Translation: BAG51182.1
AK296086 mRNA Translation: BAG58841.1
AK298347 mRNA Translation: BAG60593.1
AL117423 mRNA Translation: CAB55915.1
AC091769 Genomic DNA No translation available.
AL513185 Genomic DNA No translation available.
AL356009 Genomic DNA No translation available.
CH471083 Genomic DNA Translation: EAW54459.1
BC004190 mRNA Translation: AAH04190.1
BC004216 mRNA Translation: AAH04216.1
BC016641 mRNA Translation: AAH16641.1
AK022697 mRNA Translation: BAB14187.1 Different initiation.
Y17711 mRNA Translation: CAA76830.1 Sequence problems.
CCDSiCCDS55714.1 [Q9BPX6-4]
CCDS55715.1 [Q9BPX6-1]
PIRiT17225
RefSeqiNP_001182447.1, NM_001195518.1 [Q9BPX6-1]
NP_001182448.1, NM_001195519.1 [Q9BPX6-4]
NP_006068.2, NM_006077.3 [Q9BPX6-3]

3D structure databases

Select the link destinations:

Protein Data Bank Europe

More...
PDBei

Protein Data Bank RCSB

More...
RCSB PDBi

Protein Data Bank Japan

More...
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
4NSCX-ray3.20A/B/C/D/E/F97-476[»]
4NSDX-ray2.70A/B97-444[»]
SMRiQ9BPX6
ModBaseiSearch...
PDBe-KBiSearch...

Protein-protein interaction databases

BioGridi115646, 35 interactors
CORUMiQ9BPX6
DIPiDIP-53438N
IntActiQ9BPX6, 29 interactors
MINTiQ9BPX6
STRINGi9606.ENSP00000354415

Protein family/group databases

Allergomei3325 Hom s 4.0101
414 Hom s 4
TCDBi8.A.44.1.1 the mitochondrial ef hand ca(2+) uniporter regulator (micu) family

PTM databases

iPTMnetiQ9BPX6
PhosphoSitePlusiQ9BPX6
SwissPalmiQ9BPX6

Polymorphism and mutation databases

BioMutaiMICU1
DMDMi74761192

Proteomic databases

EPDiQ9BPX6
jPOSTiQ9BPX6
MassIVEiQ9BPX6
MaxQBiQ9BPX6
PaxDbiQ9BPX6
PeptideAtlasiQ9BPX6
PRIDEiQ9BPX6
ProteomicsDBi78588 [Q9BPX6-1]
78589 [Q9BPX6-2]
78590 [Q9BPX6-3]
78591 [Q9BPX6-4]
78592 [Q9BPX6-5]

Protocols and materials databases

The DNASU plasmid repository

More...
DNASUi
10367

Genome annotation databases

EnsembliENST00000361114; ENSP00000354415; ENSG00000107745 [Q9BPX6-1]
ENST00000398763; ENSP00000381747; ENSG00000107745 [Q9BPX6-5]
ENST00000418483; ENSP00000402470; ENSG00000107745 [Q9BPX6-4]
GeneIDi10367
KEGGihsa:10367
UCSCiuc001jtb.3 human [Q9BPX6-1]

Organism-specific databases

Comparative Toxicogenomics Database

More...
CTDi
10367
DisGeNETi10367

GeneCards: human genes, protein and diseases

More...
GeneCardsi
MICU1
HGNCiHGNC:1530 MICU1
HPAiHPA037479
HPA037480
MalaCardsiMICU1
MIMi605084 gene
615673 phenotype
neXtProtiNX_Q9BPX6
OpenTargetsiENSG00000107745
Orphaneti401768 Proximal myopathy with extrapyramidal signs
PharmGKBiPA26110

GenAtlas: human gene database

More...
GenAtlasi
Search...

Phylogenomic databases

eggNOGiKOG2643 Eukaryota
ENOG410ZSC1 LUCA
GeneTreeiENSGT00950000183079
InParanoidiQ9BPX6
KOiK22827
OrthoDBi707988at2759
PhylomeDBiQ9BPX6
TreeFamiTF313815

Enzyme and pathway databases

ReactomeiR-HSA-8949215 Mitochondrial calcium ion transport
R-HSA-8949664 Processing of SMDT1

Miscellaneous databases

ChiTaRS: a database of human, mouse and fruit fly chimeric transcripts and RNA-sequencing data

More...
ChiTaRSi
MICU1 human

The Gene Wiki collection of pages on human genes and proteins

More...
GeneWikii
CBARA1

Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens

More...
GenomeRNAii
10367
PharosiQ9BPX6

Protein Ontology

More...
PROi
PR:Q9BPX6

The Stanford Online Universal Resource for Clones and ESTs

More...
SOURCEi
Search...

Gene expression databases

BgeeiENSG00000107745 Expressed in 228 organ(s), highest expression level in cerebellar hemisphere
ExpressionAtlasiQ9BPX6 baseline and differential
GenevisibleiQ9BPX6 HS

Family and domain databases

InterProiView protein in InterPro
IPR011992 EF-hand-dom_pair
IPR018247 EF_Hand_1_Ca_BS
IPR002048 EF_hand_dom
IPR039800 MICU1/2/3
PANTHERiPTHR12294 PTHR12294, 1 hit
PfamiView protein in Pfam
PF13202 EF-hand_5, 1 hit
SMARTiView protein in SMART
SM00054 EFh, 2 hits
SUPFAMiSSF47473 SSF47473, 2 hits
PROSITEiView protein in PROSITE
PS00018 EF_HAND_1, 2 hits
PS50222 EF_HAND_2, 2 hits

ProtoNet; Automatic hierarchical classification of proteins

More...
ProtoNeti
Search...

MobiDB: a database of protein disorder and mobility annotations

More...
MobiDBi
Search...

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This subsection of the ‘Entry information’ section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiMICU1_HUMAN
<p>This subsection of the ‘Entry information’ section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called ‘Primary (citable) accession number’.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: Q9BPX6
Secondary accession number(s): A8MV96
, B3KN20, B4DJH9, B4DPI1, B5MBY3, D3YTJ3, O75785, Q9H9N6, Q9UFX0
<p>This subsection of the ‘Entry information’ section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification (‘Last modified’). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: March 18, 2008
Last sequence update: June 1, 2001
Last modified: October 16, 2019
This is version 168 of the entry and version 1 of the sequence. See complete history.
<p>This subsection of the ‘Entry information’ section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  2. SIMILARITY comments
    Index of protein domains and families
  3. Allergens
    Nomenclature of allergens and list of entries
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. Human chromosome 10
    Human chromosome 10: entries, gene names and cross-references to MIM
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