UQCR11

Summary

UQCR11 (ubiquinol-cytochrome c reductase, complex III subunit XI, HGNC:30862) is a protein-coding gene on chromosome 19p13.3, encoding Cytochrome b-c1 complex subunit 10 (O14957). Component of the ubiquinol-cytochrome c oxidoreductase, a multisubunit transmembrane complex that is part of the mitochondrial electron transport chain which drives oxidative phosphorylation.

This gene encodes the smallest known component of the ubiquinol-cytochrome c reductase complex, which forms part of the mitochondrial respiratory chain. The encoded protein may function as a binding factor for the iron-sulfur protein in this complex.

Source: NCBI Gene 10975 — RefSeq curated summary.

At a glance

• Clinical variants (ClinVar): 12 total
• MANE Select transcript: NM_006830

Identifiers

Gene identifiers

Field	Value
HGNC ID	HGNC:30862
Approved symbol	UQCR11
Name	ubiquinol-cytochrome c reductase, complex III subunit XI
Location	19p13.3
Locus type	gene with protein product
Status	Approved
Aliases	QCR10
Ensembl gene	ENSG00000127540
Ensembl biotype	protein_coding
OMIM	609711
Entrez	10975

Gene structure

Transcript identifiers

Ensembl transcripts: 10 — 8 protein_coding, 2 protein_coding_CDS_not_defined

ENST00000585671, ENST00000589880, ENST00000591899, ENST00000593029, ENST00000593264, ENST00000861759, ENST00000861760, ENST00000861761, ENST00000931862, ENST00000959495

RefSeq mRNA: 1 — MANE Select: NM_006830 NM_006830

CCDS: CCDS12073

Canonical transcript exons

ENST00000591899 — 3 exons

Exon	Start	End
ENSE00002805125	1605360	1605462
ENSE00002871477	1597169	1598215
ENSE00003522893	1599412	1599560

Expression profiles

Bgee: expression breadth ubiquitous, 218 present calls, max score 99.56.

FANTOM5 (CAGE): breadth ubiquitous, TPM avg 249.0748 / max 1296.8557, expressed in 1828 samples.

FANTOM5 promoters (1 alternative TSS)

Promoter ID	TPM avg	Samples expressed
178052	249.0748	1828

Top tissues by expression

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

Tissue	Anatomy ID	Expression score	Quality
apex of heart	UBERON:0002098	99.56	gold quality
right atrium auricular region	UBERON:0006631	99.40	gold quality
hindlimb stylopod muscle	UBERON:0004252	99.28	gold quality
mucosa of transverse colon	UBERON:0004991	99.28	gold quality
gastrocnemius	UBERON:0001388	98.82	gold quality
right adrenal gland	UBERON:0001233	98.81	gold quality
transverse colon	UBERON:0001157	98.78	gold quality
right lobe of liver	UBERON:0001114	98.77	gold quality
muscle layer of sigmoid colon	UBERON:0035805	98.76	gold quality
left adrenal gland cortex	UBERON:0035825	98.76	gold quality
left adrenal gland	UBERON:0001234	98.75	gold quality
anterior cingulate cortex	UBERON:0009835	98.74	gold quality
body of stomach	UBERON:0001161	98.70	gold quality
right adrenal gland cortex	UBERON:0035827	98.70	gold quality
lower esophagus	UBERON:0013473	98.69	gold quality
lower esophagus muscularis layer	UBERON:0035833	98.69	gold quality
esophagogastric junction muscularis propria	UBERON:0035841	98.61	gold quality
olfactory segment of nasal mucosa	UBERON:0005386	98.60	gold quality
bone marrow cell	CL:0002092	98.58	gold quality
C1 segment of cervical spinal cord	UBERON:0006469	98.55	gold quality
right frontal lobe	UBERON:0002810	98.53	gold quality
granulocyte	CL:0000094	98.52	gold quality
adenohypophysis	UBERON:0002196	98.49	gold quality
Brodmann (1909) area 9	UBERON:0013540	98.47	gold quality
metanephros cortex	UBERON:0010533	98.45	gold quality
stromal cell of endometrium	CL:0002255	98.44	gold quality
left coronary artery	UBERON:0001626	98.44	gold quality
lower esophagus mucosa	UBERON:0035834	98.38	gold quality
small intestine Peyer’s patch	UBERON:0003454	98.31	gold quality
muscle of leg	UBERON:0001383	98.27	gold quality

Single-cell (SCXA)

Detected in 15 experiment(s), a significant marker in 5.

Experiment	Marker?	Max mean expression
E-MTAB-8410	yes	50.76
E-HCAD-10	yes	32.31
E-MTAB-7316	yes	21.99
E-MTAB-10042	yes	10.77
E-MTAB-10596	no	944.24
E-MTAB-8060	no	675.88
E-MTAB-7037	no	634.93
E-HCAD-30	no	249.31
E-HCAD-6	no	41.12
E-HCAD-31	no	22.03
E-HCAD-5	no	14.62
E-HCAD-13	no	12.85
E-MTAB-10137	no	3.96
E-MTAB-9801	no	3.32
E-ANND-3	no	0.00

Regulation

Is transcription factor: no

miRNA regulators (miRDB)

34 targeting UQCR11, 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-6748-5P	100.00	65.81	1057
HSA-MIR-4425	100.00	67.59	1049
HSA-MIR-150-5P	99.99	66.69	1976
HSA-MIR-6793-5P	99.97	65.95	758
HSA-MIR-607	99.97	73.62	5593
HSA-MIR-6778-3P	99.96	67.29	2693
HSA-MIR-335-3P	99.93	73.36	4958
HSA-MIR-4753-3P	99.90	71.03	3786
HSA-MIR-153-5P	99.89	73.86	6317
HSA-MIR-3140-3P	99.88	68.47	2069
HSA-MIR-4492	99.87	68.25	3611
HSA-MIR-30E-3P	99.87	69.68	2942
HSA-MIR-34B-5P	99.78	67.56	1175
HSA-MIR-449C-5P	99.78	67.63	1168
HSA-MIR-2682-5P	99.73	67.38	1055
HSA-MIR-9851-3P	99.63	69.68	1110
HSA-MIR-4677-3P	99.49	67.91	1246
HSA-MIR-6853-3P	99.36	70.79	1558
HSA-MIR-155-5P	99.35	70.16	1509
HSA-MIR-542-3P	99.34	67.58	1270
HSA-MIR-593-5P	99.34	69.50	965
HSA-MIR-130A-5P	99.33	70.26	2623
HSA-MIR-4757-5P	99.12	64.51	981
HSA-MIR-361-5P	98.95	70.16	1340
HSA-MIR-374A-3P	98.87	67.82	1531
HSA-MIR-6829-5P	98.86	65.12	1480
HSA-MIR-4501	98.72	67.19	921
HSA-MIR-5089-5P	98.45	66.06	1388
HSA-MIR-3144-3P	98.15	67.34	677
HSA-MIR-744-3P	97.99	67.76	637

Literature-anchored findings (GeneRIF, showing 1)

• N-Acetyltransferase 10 represses Uqcr11 and Uqcrb independently of ac4C modification to promote heart regeneration. (PMID:38459019)

Cross-species orthologs

4 orthologs

Organism	Symbol	Gene ID
mus_musculus	Uqcr11	ENSMUSG00000020163
rattus_norvegicus	Uqcr11	ENSRNOG00000016952
rattus_norvegicus	Uqcr11l1	ENSRNOG00000026875
rattus_norvegicus	LOC120097699	ENSRNOG00000063903

Protein

Protein identifiers

Cytochrome b-c1 complex subunit 10 — O14957 (reviewed: O14957)

Alternative names: Complex III subunit 10, Complex III subunit XI, Ubiquinol-cytochrome c reductase complex 6.4 kDa protein

All UniProt accessions (1): O14957

UniProt curated annotations — full annotation on UniProt →

Function. Component of the ubiquinol-cytochrome c oxidoreductase, a multisubunit transmembrane complex that is part of the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over the inner membrane that drives transmembrane transport and the ATP synthase. The cytochrome b-c1 complex catalyzes electron transfer from ubiquinol to cytochrome c, linking this redox reaction to translocation of protons across the mitochondrial inner membrane, with protons being carried across the membrane as hydrogens on the quinol. In the process called Q cycle, 2 protons are consumed from the matrix, 4 protons are released into the intermembrane space and 2 electrons are passed to cytochrome c. QCR10 has a role in CIII assembly and RIP1 stability.

Subunit / interactions. Component of the ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII), a multisubunit enzyme composed of 11 subunits. The complex is composed of 3 respiratory subunits cytochrome b, cytochrome c1 and Rieske protein UQCRFS1, 2 core protein subunits UQCRC1/QCR1 and UQCRC2/QCR2, and 6 low-molecular weight protein subunits UQCRH/QCR6, UQCRB/QCR7, UQCRQ/QCR8, UQCR10/QCR9, UQCR11/QCR10 and subunit 9, the cleavage product of Rieske protein UQCRFS1. The complex exists as an obligatory dimer and forms supercomplexes (SCs) in the inner mitochondrial membrane with NADH-ubiquinone oxidoreductase (complex I, CI) and cytochrome c oxidase (complex IV, CIV), resulting in different assemblies (supercomplex SCI(1)III(2)IV(1) and megacomplex MCI(2)III(2)IV(2)).

Subcellular location. Mitochondrion inner membrane.

Similarity. Belongs to the UQCR11/QCR10 family.

RefSeq proteins (1): NP_006821* (*=MANE)

Domains & families (InterPro)

ID	Name	Type
IPR015089	UQCR	Family
IPR029027	Single_a-helix_sf	Homologous_superfamily

Pfam: PF08997

UniProt features (8 total): helix 3, topological domain 2, chain 1, transmembrane region 1, strand 1

Structure

Experimental structures (PDB)

5 structures.

PDB	Method	Resolution (Å)
9HZL	ELECTRON MICROSCOPY	2.52
9CG3	ELECTRON MICROSCOPY	2.96
5XTE	ELECTRON MICROSCOPY	3.4
5XTH	ELECTRON MICROSCOPY	3.9
5XTI	ELECTRON MICROSCOPY	17.4

Predicted structure (AlphaFold)

Model	pLDDT	Fraction very-high
AF-O14957-F1	87.97	0.50

Function

Pathways and Gene Ontology

Reactome pathways

2 pathways

ID	Pathway
R-HSA-611105	Respiratory electron transport
R-HSA-9865881	Complex III assembly

MSigDB gene sets: 172 (showing top): MODULE_93, XU_GH1_AUTOCRINE_TARGETS_UP, STARK_PREFRONTAL_CORTEX_22Q11_DELETION_DN, THEILGAARD_NEUTROPHIL_AT_SKIN_WOUND_DN, MODULE_149, GOBP_GENERATION_OF_PRECURSOR_METABOLITES_AND_ENERGY, GOBP_MITOCHONDRIAL_ELECTRON_TRANSPORT_UBIQUINOL_TO_CYTOCHROME_C, GOBP_OXIDATIVE_PHOSPHORYLATION, GOBP_ELECTRON_TRANSPORT_CHAIN, LUI_TARGETS_OF_PAX8_PPARG_FUSION, KEGG_HUNTINGTONS_DISEASE, MODULE_307, GOCC_MITOCHONDRIAL_ENVELOPE, YAO_TEMPORAL_RESPONSE_TO_PROGESTERONE_CLUSTER_13, WONG_MITOCHONDRIA_GENE_MODULE

GO Biological Process (3): generation of precursor metabolites and energy (GO:0006091), mitochondrial electron transport, ubiquinol to cytochrome c (GO:0006122), cellular respiration (GO:0045333)

GO Molecular Function (1): electron transfer activity (GO:0009055)

GO Cellular Component (4): mitochondrion (GO:0005739), mitochondrial inner membrane (GO:0005743), respiratory chain complex III (GO:0045275), membrane (GO:0016020)

Reactome top-level categories

Rollup of top-2 pathways:

Category	Pathways
Aerobic respiration and respiratory electron transport	1
Respiratory electron transport	1

GO top-level categories

Rollup of top GO terms by namespace:

Category	Terms
metabolic process	1
aerobic electron transport chain	1
mitochondrial ATP synthesis coupled electron transport	1
energy derivation by oxidation of organic compounds	1
molecular_function	1
cytoplasm	1
intracellular membrane-bounded organelle	1
organelle inner membrane	1
mitochondrial membrane	1
cytochrome complex	1
respiratory chain complex	1
transmembrane transporter complex	1
oxidoreductase complex	1
cellular anatomical structure	1

Protein interactions and networks

STRING

1154 interactions, top by confidence (×1000):

Protein A	Protein B	Partner UniProt	Score
UQCR11	COX7A1	P24310	932
UQCR11	UQCRFS1	P47985	848
UQCR11	UQCR10	Q9UDW1	833
UQCR11	UQCRH	P07919	820
UQCR11	UQCRQ	O14949	806
UQCR11	UQCRB	P14927	788
UQCR11	UQCRC1	P31930	753
UQCR11	UQCRC2	P22695	720
UQCR11	COX7B	P24311	681
UQCR11	NDUFA11	Q86Y39	679
UQCR11	COX6B1	P14854	679
UQCR11	COX5B	P10606	640
UQCR11	NDUFB1	O75438	626
UQCR11	COXFA4	O00483	619
UQCR11	MT-CYB	P00156	613
UQCR11	ATP5PF	P18859	613

IntAct

4 interactions, top by confidence:

A	B	Type	Score
UQCR11	PCNA	psi-mi:“MI:0915”(physical association)	0.370
WNT2	UQCR11	psi-mi:“MI:0915”(physical association)	0.370
UQCR11	WDR46	psi-mi:“MI:0914”(association)	0.350

BioGRID (53): UQCR11 (Synthetic Lethality), RAI14 (Affinity Capture-MS), RBM15B (Affinity Capture-MS), RBMXL2 (Affinity Capture-MS), PRRC2A (Affinity Capture-MS), ESF1 (Affinity Capture-MS), WDR46 (Affinity Capture-MS), SFSWAP (Affinity Capture-MS), DIDO1 (Affinity Capture-MS), LEMD2 (Affinity Capture-MS), RSRC1 (Affinity Capture-MS), TJP2 (Affinity Capture-MS), UTP11L (Affinity Capture-MS), YLPM1 (Affinity Capture-MS), ESCO2 (Affinity Capture-MS)

ESM2 similar proteins: A0A1D8PDP8, A0A1D8PLP3, A1XQT2, A8MTT3, G2TRT6, O14949, O14957, O74433, O75964, P07552, P09669, P13271, P22289, P37299, P42117, P50523, P56393, P80431, P80977, Q08CE7, Q1LUK1, Q28852, Q2L897, Q4R648, Q54QR8, Q5R597, Q5R9K2, Q5RFH0, Q5RK28, Q6CJX2, Q6CWK9, Q6FWE8, Q6PDU7, Q757F0, Q7SGT7, Q7TQ16, Q7YRJ9, Q7YRK0, Q7YRK2, Q7YRK3

Diamond homologs: O14957, P07552, Q9CPX8

SIGNOR signaling

1 interactions.

A	Effect	B	Mechanism
UQCR11	“form complex”	“CoQ-cytochrome c reductase-Mitochondrial respiratory chain complex III”	binding

Disease & clinical

Clinical variants and AI predictions

ClinVar

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

Classification	Count (floor)
Pathogenic	0
Likely pathogenic	0
Uncertain significance	7
Likely benign	1
Benign	0

Top pathogenic / likely-pathogenic (0)

SpliceAI

679 predictions. Top by Δscore:

Variant	Effect	Δscore
19:1598127:G:C	donor_gain	1.0000
19:1599556:GGACC:G	acceptor_gain	1.0000
19:1599557:GACC:G	acceptor_gain	1.0000
19:1599557:GACCC:G	acceptor_loss	1.0000
19:1599558:ACC:A	acceptor_gain	1.0000
19:1599558:ACCCT:A	acceptor_loss	1.0000
19:1599559:CC:C	acceptor_gain	1.0000
19:1599559:CCC:C	acceptor_gain	1.0000
19:1599560:CC:C	acceptor_gain	1.0000
19:1599561:C:A	acceptor_loss	1.0000
19:1599561:C:CC	acceptor_gain	1.0000
19:1599561:C:T	acceptor_gain	1.0000
19:1599562:T:A	acceptor_loss	1.0000
19:1597893:ATGAG:A	donor_gain	0.9900
19:1598137:T:A	donor_gain	0.9900
19:1598149:T:TA	donor_gain	0.9900
19:1599405:AACTT:A	donor_loss	0.9900
19:1599406:ACTTA:A	donor_loss	0.9900
19:1599407:CTT:C	donor_loss	0.9900
19:1599407:CTTA:C	donor_loss	0.9900
19:1599408:TTA:T	donor_loss	0.9900
19:1599409:T:TG	donor_loss	0.9900
19:1599410:A:AC	donor_gain	0.9900
19:1599410:A:C	donor_loss	0.9900
19:1599410:ACC:A	donor_loss	0.9900
19:1599411:C:CC	donor_gain	0.9900
19:1599461:G:A	donor_gain	0.9900
19:1599565:G:T	acceptor_gain	0.9900
19:1597939:T:A	donor_gain	0.9800
19:1598212:GCACC:G	acceptor_loss	0.9800

AlphaMissense

0 scored. Top likely-pathogenic:

dbSNP variants (sampled 300 via entrez): RS1000100831 (19:1604709 G>C), RS1000223771 (19:1599323 C>T), RS1000318476 (19:1606344 A>C,G), RS1000438811 (19:1601122 T>C), RS1000439712 (19:1606153 C>G,T), RS1000491153 (19:1601471 G>C), RS1000890668 (19:1599213 C>T), RS1001501922 (19:1606934 G>A), RS1001617984 (19:1601890 G>A), RS1001834544 (19:1607051 G>C), RS1001895564 (19:1598130 G>GTGGGTTTC), RS1002285598 (19:1600477 T>C), RS1002415340 (19:1602657 G>A), RS1002439167 (19:1605770 T>C), RS1002460865 (19:1605992 T>C)

Disease associations

OMIM: gene MIM:609711 | disease phenotypes:

GenCC curated gene-disease

Mondo (0):

Orphanet (0):

HPO phenotypes

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

GWAS associations

0 associations (top):

Drugs & pharmacology

Drug and pharmacology data

Is drug target: no

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

CTD chemical–gene interactions

32 total (human), top 30 by PubMed support.

Chemical	Actions (top 5)	PubMed papers
Valproic Acid	affects expression, increases expression, increases methylation	4
Acetaminophen	affects cotreatment, decreases expression	2
triphenyl phosphate	affects expression	1
tris(2-butoxyethyl) phosphate	affects expression	1
sodium arsenite	decreases expression	1
cobaltous chloride	decreases expression	1
ochratoxin A	increases expression	1
tris(chloroethyl)phosphate	increases abundance, decreases expression	1
perfluorooctane sulfonic acid	increases expression	1
CGP 52608	affects binding, increases reaction	1
perfluoro-n-nonanoic acid	increases expression	1
(+)-JQ1 compound	decreases expression	1
2,3,5-trichloro-6-phenyl-(1,4)benzoquinone	decreases expression	1
alpha-Chlorohydrin	decreases expression	1
Atrazine	decreases expression	1
Bilirubin	decreases expression	1
Cadmium	increases abundance, increases expression	1
Doxorubicin	increases expression	1
Flame Retardants	decreases expression, increases abundance	1
Fluorouracil	increases expression	1
Lipopolysaccharides	affects cotreatment, decreases expression	1
Methotrexate	affects response to substance	1
Smoke	decreases expression	1
Thiram	decreases expression	1
Tretinoin	increases expression	1
Tunicamycin	decreases expression	1
Cyclosporine	decreases expression	1
Sodium Selenite	increases expression	1
Cadmium Chloride	increases abundance, increases expression	1
Thapsigargin	decreases expression	1

Clinical trials (associated diseases)

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