MICOS10

Summary

MICOS10 (mitochondrial contact site and cristae organizing system subunit 10, HGNC:32068) is a protein-coding gene on chromosome 1p36.13, encoding MICOS complex subunit MIC10 (Q5TGZ0). Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane. It is a selective cancer dependency (DepMap: 49.3% of cell lines).

Involved in cristae formation. Located in mitochondrion. Part of MIB complex; MICOS complex; and SAM complex.

Source: NCBI Gene 440574 — RefSeq curated summary.

At a glance

• GWAS associations: 11
• Clinical variants (ClinVar): 22 total
• Druggable target: yes
• Cancer dependency (DepMap): dependent in 49.3% of screened cell lines
• MANE Select transcript: NM_001032363

Identifiers

Gene identifiers

Field	Value
HGNC ID	HGNC:32068
Approved symbol	MICOS10
Name	mitochondrial contact site and cristae organizing system subunit 10
Location	1p36.13
Locus type	gene with protein product
Status	Approved
Aliases	RP5-1056L3.2, FLJ36999, MIO10, Mic10
Ensembl gene	ENSG00000173436
Ensembl biotype	protein_coding
OMIM	616574
Entrez	440574

Gene structure

Transcript identifiers

Ensembl transcripts: 14 — 8 protein_coding_CDS_not_defined, 6 protein_coding

ENST00000322753, ENST00000462646, ENST00000467029, ENST00000479642, ENST00000481464, ENST00000485362, ENST00000486890, ENST00000498067, ENST00000498642, ENST00000617872, ENST00000648702, ENST00000917696, ENST00000917697, ENST00000917698

RefSeq mRNA: 3 — MANE Select: NM_001032363 NM_001032363, NM_001204082, NM_001204083

CCDS: CCDS30620, CCDS72719

Canonical transcript exons

ENST00000322753 — 4 exons

Exon	Start	End
ENSE00001465875	19626387	19629820
ENSE00001841013	19596979	19597109
ENSE00003582626	19623474	19623583
ENSE00003619534	19622100	19622147

Expression profiles

Bgee: expression breadth ubiquitous, 214 present calls, max score 99.20.

FANTOM5 (CAGE): breadth broad, TPM avg 0.9895 / max 87.4256, expressed in 188 samples.

FANTOM5 promoters (4 alternative TSS)

Promoter ID	TPM avg	Samples expressed
1066	63.3860	1826
1067	0.8926	162
1068	0.0863	26
1064	0.0106	4

Top tissues by expression

248 total, by Bgee expression score (0-100, higher = more expressed):

Tissue	Anatomy ID	Expression score	Quality
mucosa of transverse colon	UBERON:0004991	99.20	gold quality
apex of heart	UBERON:0002098	99.12	gold quality
right atrium auricular region	UBERON:0006631	99.03	gold quality
lower esophagus	UBERON:0013473	99.00	gold quality
lower esophagus muscularis layer	UBERON:0035833	99.00	gold quality
muscle layer of sigmoid colon	UBERON:0035805	98.90	gold quality
anterior cingulate cortex	UBERON:0009835	98.89	gold quality
esophagogastric junction muscularis propria	UBERON:0035841	98.88	gold quality
transverse colon	UBERON:0001157	98.82	gold quality
right frontal lobe	UBERON:0002810	98.72	gold quality
Brodmann (1909) area 9	UBERON:0013540	98.70	gold quality
rectum	UBERON:0001052	98.64	gold quality
colonic epithelium	UBERON:0000397	98.62	gold quality
metanephros cortex	UBERON:0010533	98.55	gold quality
body of stomach	UBERON:0001161	98.50	gold quality
adenohypophysis	UBERON:0002196	98.49	gold quality
endocervix	UBERON:0000458	98.42	gold quality
ectocervix	UBERON:0012249	98.40	gold quality
popliteal artery	UBERON:0002250	98.34	gold quality
tibial artery	UBERON:0007610	98.34	gold quality
small intestine Peyer’s patch	UBERON:0003454	98.32	gold quality
right adrenal gland	UBERON:0001233	98.26	gold quality
right adrenal gland cortex	UBERON:0035827	98.24	gold quality
lower esophagus mucosa	UBERON:0035834	98.24	gold quality
left coronary artery	UBERON:0001626	98.21	gold quality
hindlimb stylopod muscle	UBERON:0004252	98.16	gold quality
C1 segment of cervical spinal cord	UBERON:0006469	98.16	gold quality
ascending aorta	UBERON:0001496	98.15	gold quality
left adrenal gland	UBERON:0001234	98.14	gold quality
thoracic aorta	UBERON:0001515	98.13	gold quality

Single-cell (SCXA)

Detected in 4 experiment(s), a significant marker in 2.

Experiment	Marker?	Max mean expression
E-HCAD-11	yes	8.36
E-GEOD-124858	no	260.11
E-MTAB-7606	no	133.96
E-ANND-3	no	0.00

Regulation

Is transcription factor: no

miRNA regulators (miRDB)

75 targeting MICOS10, top 30 by miRDB confidence (max_score; target_count = how many genes the miRNA targets in total — lower means more specific):

miRNA	Max score	Avg score	miRNA target_count
HSA-MIR-450A-1-3P	100.00	69.33	1837
HSA-MIR-4747-5P	100.00	67.90	2681
HSA-MIR-5196-5P	100.00	67.98	2761
HSA-MIR-30A-5P	100.00	76.31	3233
HSA-MIR-30B-5P	100.00	76.29	3248
HSA-MIR-30C-5P	100.00	76.29	3248
HSA-MIR-30D-5P	100.00	76.32	3233
HSA-MIR-30E-5P	100.00	76.32	3242
HSA-MIR-3924	100.00	72.09	2394
HSA-MIR-4533	100.00	69.48	2758
HSA-MIR-499A-5P	99.98	70.79	1323
HSA-MIR-3605-5P	99.96	67.12	932
HSA-MIR-651-3P	99.94	73.48	5177
HSA-MIR-6809-3P	99.91	71.45	3814
HSA-MIR-153-5P	99.89	73.86	6317
HSA-MIR-548E-5P	99.89	72.73	4486
HSA-MIR-576-5P	99.84	70.46	2582
HSA-MIR-4668-5P	99.79	70.58	3782
HSA-MIR-4422	99.72	72.07	2908
HSA-MIR-4524A-3P	99.72	66.85	2406
HSA-MIR-1200	99.71	70.42	1838
HSA-MIR-3714	99.71	70.74	2671
HSA-MIR-29B-2-5P	99.67	68.98	1726
HSA-MIR-3202	99.66	67.70	2737
HSA-MIR-378A-5P	99.65	66.33	1311
HSA-MIR-586	99.65	70.40	2051
HSA-MIR-7156-5P	99.64	68.81	1369
HSA-MIR-1252-3P	99.55	67.71	2862
HSA-MIR-7853-5P	99.54	69.30	2055
HSA-MIR-105-5P	99.54	69.24	2060

Functional genomics

DepMap (CRISPR cell-line fitness): dependent in 49.3% of screened cell lines.

Literature-anchored findings (GeneRIF, showing 5)

• Proteomic analyses reveal MINOS1/Mio10 as a novel constituent of Mitofilin/Fcj1 complexes in human and yeast mitochondria (PMID:22114354)
• APOOL is a cardiolipin-binding component of the Mitofilin/MINOS protein complex. (PMID:23704930)
• Listeria monocytogenes Exploits Mitochondrial Contact Site and Cristae Organizing System Complex Subunit Mic10 To Promote Mitochondrial Fragmentation and Cellular Infection. (PMID:32019800)
• Multicolor 3D MINFLUX nanoscopy of mitochondrial MICOS proteins. (PMID:32788360)
• Conserved GxxxG and WN motifs of MIC13 are essential for bridging two MICOS subcomplexes. (PMID:34271005)

Cross-species orthologs

2 orthologs

Organism	Symbol	Gene ID
mus_musculus	Micos10	ENSMUSG00000050608
rattus_norvegicus	Micos10	ENSRNOG00000089574

Protein

Protein identifiers

MICOS complex subunit MIC10 — Q5TGZ0 (reviewed: Q5TGZ0)

Alternative names: Mitochondrial inner membrane organizing system protein 1

All UniProt accessions (3): Q5TGZ0, A0A087WU07, A0A3B3IRY5

UniProt curated annotations — full annotation on UniProt →

Function. Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane.

Subunit / interactions. Component of the mitochondrial contact site and cristae organizing system (MICOS) complex, composed of at least MICOS10/MIC10, CHCHD3/MIC19, CHCHD6/MIC25, APOOL/MIC27, IMMT/MIC60, APOO/MIC23/MIC26 and MICOS13/MIC13. This complex was also known under the names MINOS or MitOS complex. The MICOS complex associates with mitochondrial outer membrane proteins SAMM50, MTX1 and MTX2 (together described as components of the mitochondrial outer membrane sorting assembly machinery (SAM) complex) and DNAJC11, mitochondrial inner membrane protein TMEM11 and with HSPA9. The MICOS and SAM complexes together with DNAJC11 are part of a large protein complex spanning both membranes termed the mitochondrial intermembrane space bridging (MIB) complex. Interacts with IMMT/MIC60 and MICOS13/MIC13. Interacts with APOO/MIC23/MIC26 and APOOL/MIC27. Interacts with ARMC1.

Subcellular location. Mitochondrion inner membrane.

Similarity. Belongs to the MICOS complex subunit Mic10 family.

Isoforms (2)

UniProt ID	Names	Canonical?
Q5TGZ0-1	1	yes
Q5TGZ0-2	2

RefSeq proteins (3): NP_001027535, NP_001191011, NP_001191012 (=MANE)

Domains & families (InterPro)

ID	Name	Type
IPR007512	Mic10	Family

Pfam: PF04418

UniProt features (7 total): splice variant 2, initiator methionine 1, chain 1, transmembrane region 1, topological domain 1, modified residue 1

Structure

Experimental structures (PDB)

0 structures.

Predicted structure (AlphaFold)

Model	pLDDT	Fraction very-high
AF-Q5TGZ0-F1	72.13	0.00

Functional residue map

Curated UniProt residues grouped by drug-discovery relevance — catalytic, ligand-binding, modification, and mutation-validated positions. Source: UniProtKB sequence features.

Post-translational modifications (1): 2

Function

Pathways and Gene Ontology

Reactome pathways

3 pathways

ID	Pathway
R-HSA-8949613	Cristae formation
R-HSA-1592230	Mitochondrial biogenesis
R-HSA-1852241	Organelle biogenesis and maintenance

MSigDB gene sets: 141 (showing top): STARK_PREFRONTAL_CORTEX_22Q11_DELETION_DN, GOBP_CRISTAE_FORMATION, GOBP_INNER_MITOCHONDRIAL_MEMBRANE_ORGANIZATION, GOCC_MITOCHONDRIAL_ENVELOPE, LASTOWSKA_NEUROBLASTOMA_COPY_NUMBER_DN, GOBP_MEMBRANE_ORGANIZATION, ACEVEDO_METHYLATED_IN_LIVER_CANCER_DN, NAKAMURA_TUMOR_ZONE_PERIPHERAL_VS_CENTRAL_UP, GOCC_ORGANELLE_MEMBRANE_CONTACT_SITE, GOCC_OUTER_MITOCHONDRIAL_MEMBRANE_PROTEIN_COMPLEX, GOCC_ORGANELLE_INNER_MEMBRANE, GOCC_INNER_MITOCHONDRIAL_MEMBRANE_PROTEIN_COMPLEX, GOCC_MEMBRANE_PROTEIN_COMPLEX, GOCC_MITOCHONDRIAL_PROTEIN_CONTAINING_COMPLEX, RAY_TUMORIGENESIS_BY_ERBB2_CDC25A_DN

GO Biological Process (2): inner mitochondrial membrane organization (GO:0007007), cristae formation (GO:0042407)

GO Molecular Function (1): protein binding (GO:0005515)

GO Cellular Component (7): SAM complex (GO:0001401), mitochondrion (GO:0005739), mitochondrial inner membrane (GO:0005743), mitochondrial crista junction (GO:0044284), MICOS complex (GO:0061617), MIB complex (GO:0140275), membrane (GO:0016020)

Reactome top-level categories

Rollup of top-2 pathways:

Category	Pathways
Mitochondrial biogenesis	1
Organelle biogenesis and maintenance	1

GO top-level categories

Rollup of top GO terms by namespace:

Category	Terms
inner mitochondrial membrane protein complex	2
mitochondrial membrane organization	1
inner mitochondrial membrane organization	1
binding	1
mitochondrial outer membrane translocase complex	1
cytoplasm	1
intracellular membrane-bounded organelle	1
organelle inner membrane	1
mitochondrial membrane	1
mitochondrial inner membrane	1
organelle membrane contact site	1
cellular anatomical structure	1

Protein interactions and networks

STRING

1030 interactions, top by confidence (×1000):

Protein A	Protein B	Partner UniProt	Score
MICOS10	CHCHD3	Q9NX63	994
MICOS10	CHCHD6	Q9BRQ6	982
MICOS10	IMMT	Q16891	975
MICOS10	APOOL	Q6UXV4	965
MICOS10	APOO	Q9BUR5	939
MICOS10	MICOS13	Q5XKP0	912
MICOS10	SAMM50	Q9Y512	766
MICOS10	MTX1	Q13505	697
MICOS10	MTX2	O75431	667
MICOS10	DNAJC11	Q9NVH1	629
MICOS10	ELOF1	P60002	587
MICOS10	CHCHD10	Q8WYQ3	574
MICOS10	MRPL48	Q96GC5	543
MICOS10	ZNF780A	O75290	513
MICOS10	GUK1	Q16774	490

IntAct

12 interactions, top by confidence:

A	B	Type	Score
MICOS10	APOC4	psi-mi:“MI:0915”(physical association)	0.560
MPC2	MICOS10	psi-mi:“MI:0915”(physical association)	0.560
APOC4	MICOS10	psi-mi:“MI:0915”(physical association)	0.560
MICOS10	CIDEB	psi-mi:“MI:0915”(physical association)	0.560
SLC6A3	GET1	psi-mi:“MI:0914”(association)	0.350
SVOP	PGRMC1	psi-mi:“MI:0914”(association)	0.350
MICOS10	MPC2	psi-mi:“MI:0915”(physical association)	0.000
MICOS10	CIDEB	psi-mi:“MI:0915”(physical association)	0.000

BioGRID (92): MINOS1 (Affinity Capture-RNA), MINOS1 (Affinity Capture-RNA), MINOS1 (Affinity Capture-MS), MINOS1 (Affinity Capture-RNA), MINOS1 (Affinity Capture-RNA), MINOS1 (Affinity Capture-RNA), MINOS1 (Negative Genetic), MINOS1 (Negative Genetic), MINOS1 (Positive Genetic), MINOS1 (Positive Genetic), MINOS1 (Positive Genetic), MINOS1 (Positive Genetic), MINOS1 (Negative Genetic), MINOS1 (Negative Genetic), MINOS1 (Negative Genetic)

ESM2 similar proteins: A0A1D8PI78, A1XQR6, A2RVP7, A4QNF3, B2RYW8, O44477, O48528, P0CR88, P0CR89, P25710, P32897, P60602, P60603, P63030, P63031, P79082, P87130, P87146, Q02889, Q12328, Q3SZV8, Q3ZCG2, Q4V7T9, Q54K35, Q54QM0, Q55GU4, Q5NVQ1, Q5TGZ0, Q6BT35, Q6BZY4, Q6CRJ6, Q6FT37, Q6NYD1, Q751T2, Q75E80, Q7KSC4, Q7TNS2, Q80W89, Q8HXG6, Q8IN78

Diamond homologs: B2RYW8, Q5TGZ0, Q751T2, Q7RYI0, Q7TNS2, Q8LAJ9, Q96VH5, Q9HFF0, Q9N4K0

SIGNOR signaling

0 interactions.

Disease & clinical

Clinical variants and AI predictions

ClinVar

22 variants total. Per-class counts are floors (≥ shown; pagination cap):

Classification	Count (floor)
Pathogenic	0
Likely pathogenic	0
Uncertain significance	20
Likely benign	2
Benign	0

Top pathogenic / likely-pathogenic (0)

SpliceAI

1206 predictions. Top by Δscore:

Variant	Effect	Δscore
1:19597105:GATAG:G	donor_gain	1.0000
1:19597106:A:G	donor_gain	1.0000
1:19597106:ATAGG:A	donor_loss	1.0000
1:19597108:AGGT:A	donor_loss	1.0000
1:19597109:GGTAA:G	donor_loss	1.0000
1:19597110:GTAAG:G	donor_loss	1.0000
1:19597111:T:A	donor_loss	1.0000
1:19622148:G:GG	donor_gain	1.0000
1:19626474:GGA:G	donor_gain	1.0000
1:19626475:GAG:G	donor_gain	1.0000
1:19626477:G:GG	donor_gain	1.0000
1:19597069:G:GT	donor_gain	0.9900
1:19597110:G:GG	donor_gain	0.9900
1:19623579:TCA:T	donor_gain	0.9900
1:19623584:G:GG	donor_gain	0.9900
1:19626474:G:GT	donor_gain	0.9900
1:19626475:GA:G	donor_gain	0.9900
1:19485430:CTTTA:C	donor_loss	0.9800
1:19485431:TTTA:T	donor_loss	0.9800
1:19485432:TTA:T	donor_loss	0.9800
1:19485433:TACCA:T	donor_loss	0.9800
1:19485434:A:AC	donor_gain	0.9800
1:19485435:C:CC	donor_gain	0.9800
1:19597924:TC:T	donor_gain	0.9800
1:19626464:G:GT	donor_gain	0.9800
1:19626509:A:AG	donor_gain	0.9800
1:19626510:G:GG	donor_gain	0.9800
1:19626513:A:AG	donor_gain	0.9800
1:19626457:G:GT	donor_gain	0.9700
1:19626498:ACAAT:A	donor_gain	0.9700

AlphaMissense

501 scored. Top likely-pathogenic:

Variant	Protein change	am_pathogenicity
1:19622124:T:A	V30D	0.997
1:19597082:T:C	C13R	0.996
1:19622100:G:A	G22D	0.996
1:19623504:G:A	G48D	0.996
1:19623534:G:A	C58Y	0.996
1:19597109:G:C	G22R	0.995
1:19622111:G:A	G26R	0.995
1:19622111:G:C	G26R	0.995
1:19622112:G:A	G26E	0.995
1:19622117:G:A	G28R	0.995
1:19622117:G:C	G28R	0.995
1:19622118:G:A	G28E	0.995
1:19623482:T:A	W41R	0.995
1:19623482:T:C	W41R	0.995
1:19623516:G:A	G52E	0.995
1:19623533:T:A	C58S	0.995
1:19623533:T:C	C58R	0.995
1:19623534:G:C	C58S	0.995
1:19597084:T:G	C13W	0.994
1:19597083:G:A	C13Y	0.993
1:19623498:G:A	G46D	0.993
1:19623535:T:G	C58W	0.993
1:19622106:G:A	G24D	0.992
1:19623509:G:A	G50R	0.992
1:19623509:G:C	G50R	0.992
1:19623515:G:A	G52R	0.991
1:19623515:G:C	G52R	0.991
1:19597082:T:A	C13S	0.990
1:19597083:G:C	C13S	0.990
1:19622105:G:C	G24R	0.990

dbSNP variants (sampled 300 via entrez): RS1000068305 (1:19595625 A>G), RS1000116952 (1:19596053 T>C), RS1000170163 (1:19603599 T>A), RS1000280616 (1:19597670 C>G,T), RS1000492563 (1:19621320 A>C,G), RS1000552537 (1:19596305 T>C), RS1000563156 (1:19602682 A>G,T), RS1000669162 (1:19596824 G>A), RS1000884736 (1:19621058 C>G,T), RS1000982177 (1:19614438 C>G,T), RS1001114677 (1:19602566 A>G), RS1001128239 (1:19608416 A>C,G,T), RS1001463754 (1:19615090 T>C), RS1001554026 (1:19598687 C>T), RS1001606671 (1:19626326 T>C)

Disease associations

OMIM: gene MIM:616574 | disease phenotypes:

GenCC curated gene-disease

Mondo (0):

Orphanet (0):

HPO phenotypes

0 total (0 of 0 shown, HPO-id order):

GWAS associations

11 associations (top):

Study	Trait	p-value
GCST002458_2	Serum thyroid-stimulating hormone levels	3.000000e-07
GCST003988_22	Hypothyroidism	8.000000e-09
GCST006309_2	Post bronchodilator percent predicted FEV1 in smoking	9.000000e-06
GCST010696_18	Cortical thickness (min-P)	1.000000e-10
GCST010697_49	Cortical surface area (min-P)	9.000000e-13
GCST010698_58	Subcortical volume (min-P)	9.000000e-10
GCST010699_28	Brain morphology (min-P)	3.000000e-09
GCST010700_3	Cortical thickness (MOSTest)	2.000000e-20
GCST010701_23	Cortical surface area (MOSTest)	2.000000e-44
GCST010702_68	Subcortical volume (MOSTest)	2.000000e-11
GCST010703_80	Brain morphology (MOSTest)	3.000000e-16

EFO canonical traits (3, from GWAS)

EFO ID	Trait name
EFO:0004314	forced expiratory volume
EFO:0004346	neuroimaging measurement
EFO:0004840	cortical thickness

Drugs & pharmacology

Drug and pharmacology data

Is drug target: yes

ChEMBL targets (1): CHEMBL4105956 (SINGLE PROTEIN)

PharmGKB: 1 entry (VIP=true, CPIC=false)

CTD chemical–gene interactions

19 total (human), top 19 by PubMed support.

Chemical	Actions (top 5)	PubMed papers
Valproic Acid	affects expression, increases expression	4
Benzo(a)pyrene	increases expression	3
bisphenol A	increases abundance, decreases expression, affects cotreatment, affects expression	2
ginger extract	affects cotreatment, affects expression, increases abundance	1
dicrotophos	decreases expression	1
arsenite	affects binding, increases reaction	1
sodium arsenite	increases expression	1
K 7174	decreases expression	1
Sunitinib	increases expression	1
Acetaminophen	increases expression	1
Air Pollutants	decreases expression, increases abundance	1
Calcitriol	increases expression, affects cotreatment	1
Doxorubicin	decreases expression	1
Oils, Volatile	affects expression, increases abundance, affects cotreatment	1
Smoke	decreases expression	1
Dihydrotestosterone	increases expression	1
Testosterone	affects cotreatment, increases expression	1
Urethane	decreases expression	1
Particulate Matter	decreases expression, increases abundance	1

ChEMBL screening assays

1 unique, capped per target: 1 binding

Representative assays (with source publication via chembl_document):

Assay ID	Type	Description	Source paper
CHEMBL4012556	Binding	Binding affinity to MICOS complex subunit MIC10 in human INA-6 cells after 3 hrs by nanoLC-MS/MS method	Ugi Reaction-Derived α-Acyl Aminocarboxamides Bind to Phosphatidylinositol 3-Kinase-Related Kinases, Inhibit HSF1-Dependent Heat Shock Response, and Induce Apoptosis in Multiple Myeloma Cells. — J Med Chem

Cellosaurus cell lines

1 cell lines: 1 cancer cell line

First 10 cell lines (id-ordered, not curated):

Cellosaurus	Name	Category	Sex
CVCL_SY39	HAP1 MINOS1 (-)	Cancer cell line	Male

Clinical trials (associated diseases)

0 trials via MONDO — disease-level, not drug-specific.