COPRS

Summary

COPRS (coordinator of PRMT5 and differentiation stimulator, HGNC:28848) is a protein-coding gene on chromosome 17q11.2, encoding Coordinator of PRMT5 and differentiation stimulator (Q9NQ92). Histone-binding protein required for histone H4 methyltransferase activity of PRMT5.

Enables histone binding activity. Involved in chromatin remodeling. Located in cytosol; nucleoplasm; and plasma membrane.

Source: NCBI Gene 55352 — RefSeq curated summary.

At a glance

• GWAS associations: 7
• Clinical variants (ClinVar): 41 total — 4 pathogenic
• MANE Select transcript: NM_018405

Identifiers

Gene identifiers

Field	Value
HGNC ID	HGNC:28848
Approved symbol	COPRS
Name	coordinator of PRMT5 and differentiation stimulator
Location	17q11.2
Locus type	gene with protein product
Status	Approved
Aliases	TTP1, HSA272196, COPR5
Ensembl gene	ENSG00000172301
Ensembl biotype	protein_coding
Entrez	55352

Gene structure

Transcript identifiers

Ensembl transcripts: 5 — 3 protein_coding, 2 nonsense_mediated_decay

ENST00000302362, ENST00000378634, ENST00000496655, ENST00000579741, ENST00000579984

RefSeq mRNA: 2 — MANE Select: NM_018405 NM_001330176, NM_018405

CCDS: CCDS11268, CCDS82100

Canonical transcript exons

ENST00000302362 — 4 exons

Exon	Start	End
ENSE00002720400	31859101	31859244
ENSE00003496226	31851871	31852308
ENSE00003496990	31852812	31853030
ENSE00003609489	31856799	31856865

Expression profiles

Bgee: expression breadth ubiquitous, 259 present calls, max score 99.24.

FANTOM5 (CAGE): breadth ubiquitous, TPM avg 42.7993 / max 225.0152, expressed in 1820 samples.

FANTOM5 promoters (8 alternative TSS)

Promoter ID	TPM avg	Samples expressed
165245	30.6078	1808
165246	7.5552	1723
165244	3.2722	1250
165247	1.2479	779
165242	0.0480	3
165243	0.0386	4
165240	0.0211	5
165241	0.0083	4

Top tissues by expression

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

Tissue	Anatomy ID	Expression score	Quality
left testis	UBERON:0004533	99.24	gold quality
right testis	UBERON:0004534	99.16	gold quality
adult organism	UBERON:0007023	98.76	gold quality
oocyte	CL:0000023	98.59	gold quality
hypothalamus	UBERON:0001898	98.07	gold quality
secondary oocyte	CL:0000655	98.04	gold quality
anterior cingulate cortex	UBERON:0009835	97.94	gold quality
Brodmann (1909) area 9	UBERON:0013540	97.81	gold quality
sperm	CL:0000019	97.76	gold quality
right frontal lobe	UBERON:0002810	97.75	gold quality
nucleus accumbens	UBERON:0001882	97.59	gold quality
left ventricle myocardium	UBERON:0006566	97.59	gold quality
amygdala	UBERON:0001876	97.58	gold quality
putamen	UBERON:0001874	97.56	gold quality
testis	UBERON:0000473	97.53	gold quality
prefrontal cortex	UBERON:0000451	97.52	gold quality
caudate nucleus	UBERON:0001873	97.28	gold quality
kidney epithelium	UBERON:0004819	97.23	gold quality
dorsolateral prefrontal cortex	UBERON:0009834	97.22	gold quality
frontal cortex	UBERON:0001870	97.08	gold quality
ascending aorta	UBERON:0001496	97.03	gold quality
cardiac muscle of right atrium	UBERON:0003379	97.01	gold quality
thoracic aorta	UBERON:0001515	97.00	gold quality
muscle layer of sigmoid colon	UBERON:0035805	96.99	gold quality
descending thoracic aorta	UBERON:0002345	96.96	gold quality
neocortex	UBERON:0001950	96.94	gold quality
substantia nigra	UBERON:0002038	96.75	gold quality
right coronary artery	UBERON:0001625	96.67	gold quality
aorta	UBERON:0000947	96.65	gold quality
pons	UBERON:0000988	96.61	gold quality

Single-cell (SCXA)

Detected in 1 experiment(s), a significant marker in 1.

Experiment	Marker?	Max mean expression
E-ANND-3	yes	8.02

Regulation

Is transcription factor: no

miRNA regulators (miRDB)

15 targeting COPRS, 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-8485	100.00	77.57	4731
HSA-MIR-520D-5P	99.98	73.34	4883
HSA-MIR-524-5P	99.98	73.43	4882
HSA-MIR-141-3P	99.94	72.79	2421
HSA-MIR-200A-3P	99.94	72.68	2420
HSA-MIR-552-5P	99.93	68.56	1583
HSA-MIR-6809-3P	99.91	71.45	3814
HSA-MIR-4753-3P	99.90	71.03	3786
HSA-MIR-5695	99.41	67.48	1047
HSA-MIR-4463	98.56	66.05	1071
HSA-MIR-12120	98.05	68.44	1768
HSA-MIR-1306-5P	97.11	64.04	755
HSA-MIR-6760-3P	96.35	68.31	1001
HSA-MIR-4524B-3P	95.52	64.12	964
HSA-MIR-4330	95.44	66.39	993

Cross-species orthologs

2 orthologs

Organism	Symbol	Gene ID
mus_musculus	Coprs	ENSMUSG00000031458
rattus_norvegicus	Coprs	ENSRNOG00000000111

Protein

Protein identifiers

Coordinator of PRMT5 and differentiation stimulator — Q9NQ92 (reviewed: Q9NQ92)

Alternative names: Cooperator of PRMT5, Protein TTP1

All UniProt accessions (5): Q9NQ92, H9KV77, J3QRX4, J3QRX6, J3QRX9

UniProt curated annotations — full annotation on UniProt →

Function. Histone-binding protein required for histone H4 methyltransferase activity of PRMT5. Specifically required for histone H4 ‘Arg-3’ methylation mediated by PRMT5, but not histone H3 ‘Arg-8’ methylation, suggesting that it modulates the substrate specificity of PRMT5. Specifically interacts with the N-terminus of histone H4 but not with histone H3, suggesting that it acts by promoting the association between histone H4 and PRMT5. Involved in CCNE1 promoter repression. Plays a role in muscle cell differentiation by modulating the recruitment of PRMT5 to the promoter of genes involved in the coordination between cell cycle exit and muscle differentiation.

Subunit / interactions. Interacts with PRMT5. Interacts with histone H4; specifically interacts with the N-terminus of histone H4 but not with histone H3. Interacts with CBFB. Found in a complex with PRMT5, RUNX1 and CBFB.

Subcellular location. Nucleus.

RefSeq proteins (2): NP_001317105, NP_060875* (*=MANE)

Domains & families (InterPro)

ID	Name	Type
IPR029289	COPR5	Family

Pfam: PF15340

UniProt features (9 total): compositionally biased region 3, modified residue 3, chain 1, region of interest 1, sequence variant 1

Structure

Experimental structures (PDB)

0 structures.

Predicted structure (AlphaFold)

Model	pLDDT	Fraction very-high
AF-Q9NQ92-F1	62.95	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 (3): 1, 66, 75

Function

Pathways and Gene Ontology

Reactome pathways

1 pathways

ID	Pathway
R-HSA-3214858	RMTs methylate histone arginines

MSigDB gene sets: 131 (showing top): GOBP_RIBOSOME_BIOGENESIS, STARK_PREFRONTAL_CORTEX_22Q11_DELETION_DN, chr17q11, GOBP_MUSCLE_STRUCTURE_DEVELOPMENT, GOBP_CHROMATIN_REMODELING, GOBP_RIBONUCLEOPROTEIN_COMPLEX_BIOGENESIS, NAKAMURA_TUMOR_ZONE_PERIPHERAL_VS_CENTRAL_UP, GOCC_TRANSFERASE_COMPLEX, LEIN_MIDBRAIN_MARKERS, LEIN_PONS_MARKERS, GOCC_METHYLTRANSFERASE_COMPLEX, GOCC_METHYLOSOME, GOMF_HISTONE_BINDING, ER_Q6_02, COLDREN_GEFITINIB_RESISTANCE_UP

GO Biological Process (3): chromatin remodeling (GO:0006338), muscle organ development (GO:0007517), chromatin organization (GO:0006325)

GO Molecular Function (2): histone binding (GO:0042393), protein binding (GO:0005515)

GO Cellular Component (4): nucleus (GO:0005634), nucleoplasm (GO:0005654), cytosol (GO:0005829), plasma membrane (GO:0005886)

Reactome top-level categories

Rollup of top-1 pathways:

Category	Pathways
Chromatin modifying enzymes	1

GO top-level categories

Rollup of top GO terms by namespace:

Category	Terms
cellular anatomical structure	2
chromatin organization	1
animal organ development	1
muscle structure development	1
cellular component organization	1
protein binding	1
binding	1
intracellular membrane-bounded organelle	1
nuclear lumen	1
cytoplasm	1
membrane	1
cell periphery	1

Protein interactions and networks

STRING

610 interactions, top by confidence (×1000):

Protein A	Protein B	Partner UniProt	Score
COPRS	PRMT5	O14744	912
COPRS	CRLF3	Q8IUI8	693
COPRS	RAB11FIP4	Q86YS3	674
COPRS	UTP6	Q9NYH9	669
COPRS	ATAD5	Q96QE3	667
COPRS	ADAP2	Q9NPF8	636
COPRS	TEFM	Q96QE5	635
COPRS	RIOK1	Q9BRS2	635
COPRS	WDR77	Q9BQA1	631
COPRS	LRRC37B	Q96QE4	623
COPRS	COPZ1	P61923	621
COPRS	EVI2B	P34910	618
COPRS	H4C7	Q99525	580
COPRS	EVI2A	P22794	579
COPRS	H4C16	P02304	578

IntAct

82 interactions, top by confidence:

A	B	Type	Score
LSM3	LSM1	psi-mi:“MI:0914”(association)	0.950
LSM6	LSM1	psi-mi:“MI:0914”(association)	0.840
COPRS	PRMT5	psi-mi:“MI:0914”(association)	0.770
COPRS	PRMT5	psi-mi:“MI:0915”(physical association)	0.770
PRMT5	COPRS	psi-mi:“MI:0915”(physical association)	0.770
PRMT5	COPRS	psi-mi:“MI:0403”(colocalization)	0.770
NS	PIK3R2	psi-mi:“MI:0914”(association)	0.750
SNRPB	PRMT5	psi-mi:“MI:0914”(association)	0.670
VBP1	PFDN6	psi-mi:“MI:0914”(association)	0.640
SNRPB	SART1	psi-mi:“MI:0914”(association)	0.640
LACTB	CCDC102A	psi-mi:“MI:0914”(association)	0.560
RPS2	MPHOSPH10	psi-mi:“MI:0914”(association)	0.530
LSM6	PRMT5	psi-mi:“MI:0914”(association)	0.530
SNRPN	PRMT5	psi-mi:“MI:0914”(association)	0.530
CIRBP	PRMT5	psi-mi:“MI:0914”(association)	0.530
FBL	ZNF316	psi-mi:“MI:0914”(association)	0.530
EPB41L3	AP3B1	psi-mi:“MI:0914”(association)	0.530
GAR1	PRMT5	psi-mi:“MI:0914”(association)	0.530
HNRNPH2	PLOD2	psi-mi:“MI:0914”(association)	0.530
LSM4	PRMT5	psi-mi:“MI:0914”(association)	0.530

BioGRID (104): COPRS (Affinity Capture-RNA), COPRS (Affinity Capture-RNA), COPRS (Affinity Capture-RNA), COPRS (Affinity Capture-MS), COPRS (Affinity Capture-MS), COPRS (Affinity Capture-MS), COPRS (Affinity Capture-MS), COPRS (Affinity Capture-MS), COPRS (Two-hybrid), PRMT5 (Reconstituted Complex), PRMT5 (Affinity Capture-Western), PRMT5 (Co-fractionation), HIST3H3 (Reconstituted Complex), COPRS (Proximity Label-MS), COPRS (Co-purification)

ESM2 similar proteins: A0A1L8I316, A0A1W2PR82, A0A286YDK6, A2A9F4, A5PJD3, A6H7B4, A6NHS1, A6NJJ6, A6QP24, A6QPM6, D4AAA5, O08664, O43151, P24097, Q0VD86, Q1LZ80, Q1RMQ5, Q28CW2, Q2KIL8, Q32LI3, Q3B8N5, Q3T0X9, Q5M831, Q5M865, Q66MI6, Q6AY88, Q6DJE5, Q6PKN7, Q80VY2, Q8BII1, Q8C1R3, Q8N2Y8, Q8TAP8, Q8VC23, Q8VHZ9, Q90932, Q99L02, Q9BSI4, Q9BSJ6, Q9BTK6

Diamond homologs: A5PJD3, Q9CQ13, Q9NQ92

SIGNOR signaling

0 interactions.

Enriched among interaction partners

Reactome pathways and GO biological processes over-represented among this gene’s 85 IntAct physical interaction partners (hypergeometric vs the genome-wide background, BH-FDR, gene-set size 15–500, ranked by fold). A functional readout of the neighbourhood — distinct from this gene’s own memberships above, and biased toward well-studied / hub proteins, so read it as themes rather than proof.

Reactome pathways:

Pathway	Partners	Fold	FDR
Processing of Capped Intron-Containing Pre-mRNA	11	14.3×	4e-08
mRNA Splicing	8	13.9×	1e-05
mRNA Splicing - Major Pathway	12	10.4×	2e-07
Metabolism of RNA	14	9.3×	4e-08
mRNA Polyadenylation	6	8.4×	5e-03

GO biological processes:

GO term	Partners	Fold	FDR
spliceosomal snRNP assembly	6	47.8×	4e-07
mRNA splicing, via spliceosome	12	15.1×	1e-08
RNA splicing	11	13.3×	2e-07

Disease & clinical

Clinical variants and AI predictions

ClinVar

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

Classification	Count (floor)
Pathogenic	4
Likely pathogenic	0
Uncertain significance	31
Likely benign	0
Benign	0

Top pathogenic / likely-pathogenic (4)

Variant ID	HGVS	Classification
3362875	Single allele	Pathogenic
58139	GRCh38/hg38 17q11.2(chr17:30667972-32023858)x3	Pathogenic
815924	GRCh37/hg19 17q11.2(chr17:29373189-30412788)x1	Pathogenic
929369	GRCh37/hg19 17q11.2(chr17:29393104-30427403)x1	Pathogenic

SpliceAI

635 predictions. Top by Δscore:

Variant	Effect	Δscore
17:31852807:CCTA:C	donor_loss	1.0000
17:31852808:CTACC:C	donor_loss	1.0000
17:31852809:TAC:T	donor_loss	1.0000
17:31852810:A:AT	donor_loss	1.0000
17:31853027:CGGG:C	acceptor_gain	1.0000
17:31859049:C:A	donor_gain	1.0000
17:31852806:ACCT:A	donor_loss	0.9900
17:31852810:A:AC	donor_gain	0.9900
17:31852811:C:CC	donor_gain	0.9900
17:31852822:C:A	donor_gain	0.9900
17:31853026:ACGGG:A	acceptor_gain	0.9900
17:31853027:CGGGC:C	acceptor_gain	0.9900
17:31853029:GG:G	acceptor_gain	0.9900
17:31853031:C:CC	acceptor_gain	0.9900
17:31853031:CTA:C	acceptor_loss	0.9900
17:31853032:T:C	acceptor_loss	0.9900
17:31853036:T:TC	acceptor_gain	0.9900
17:31856746:T:TA	donor_gain	0.9900
17:31856861:CCAGC:C	acceptor_gain	0.9900
17:31856862:CAGC:C	acceptor_gain	0.9900
17:31856862:CAGCC:C	acceptor_gain	0.9900
17:31856865:CCT:C	acceptor_loss	0.9900
17:31856866:C:CC	acceptor_gain	0.9900
17:31856867:T:A	acceptor_loss	0.9900
17:31859048:T:TA	donor_gain	0.9900
17:31859095:GCTCA:G	donor_loss	0.9900
17:31859096:CTCA:C	donor_loss	0.9900
17:31859097:TCACC:T	donor_loss	0.9900
17:31859098:CA:C	donor_loss	0.9900
17:31859099:A:C	donor_loss	0.9900

AlphaMissense

1222 scored. Top likely-pathogenic:

Variant	Protein change	am_pathogenicity
17:31852157:A:C	F179L	0.998
17:31852157:A:T	F179L	0.998
17:31852159:A:G	F179L	0.998
17:31852158:A:G	F179S	0.993
17:31852158:A:C	F179C	0.992
17:31852849:C:A	W116C	0.991
17:31852849:C:G	W116C	0.991
17:31852172:A:C	F174L	0.986
17:31852172:A:T	F174L	0.986
17:31852174:A:G	F174L	0.986
17:31852159:A:C	F179V	0.984
17:31852851:A:G	W116R	0.984
17:31852851:A:T	W116R	0.984
17:31852159:A:T	F179I	0.983
17:31852160:C:A	Q178H	0.980
17:31852160:C:G	Q178H	0.980
17:31852153:C:G	D181H	0.978
17:31852164:C:T	G177E	0.977
17:31852216:G:C	H160D	0.977
17:31852152:T:A	D181V	0.976
17:31852213:G:C	H161D	0.976
17:31852152:T:G	D181A	0.973
17:31852164:C:A	G177V	0.973
17:31852151:A:C	D181E	0.971
17:31852151:A:T	D181E	0.971
17:31852149:G:T	A182D	0.970
17:31852165:C:G	G177R	0.969
17:31852165:C:T	G177R	0.969
17:31852211:A:C	H161Q	0.967
17:31852211:A:T	H161Q	0.967

dbSNP variants (sampled 300 via entrez): RS1000338340 (17:31856162 C>CCATA), RS1000470966 (17:31861181 G>A), RS1000603025 (17:31860975 A>G), RS1000945528 (17:31857489 T>G), RS1001148725 (17:31854059 G>A), RS1001279942 (17:31857031 T>C), RS1001315448 (17:31857201 T>C), RS1001378027 (17:31857983 AAGG>A), RS1001724363 (17:31860344 T>C), RS1002043979 (17:31855196 T>A), RS1002141571 (17:31859481 G>A,T), RS1002277395 (17:31858644 G>A,C), RS1002422644 (17:31852692 G>A,C), RS1002778135 (17:31853041 A>G), RS1003395254 (17:31859104 C>A,G,T)

Disease associations

OMIM: gene `` | disease phenotypes: MIM:613675

GenCC curated gene-disease

Mondo (1): chromosome 17q11.2 deletion syndrome, 1.4Mb (MONDO:0013357)

Orphanet (4): 17q11.2 microduplication syndrome (Orphanet:139474), Neurofibromatosis type 1 (Orphanet:636), 17q11 microdeletion syndrome (Orphanet:97685), Overgrowth-macrocephaly-facial dysmorphism syndrome (Orphanet:137634)

HPO phenotypes

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

GWAS associations

7 associations (top):

Study	Trait	p-value
GCST006993_13	Hippocampal volume in Alzheimer’s disease dementia	7.000000e-07
GCST007269_162	Pulse pressure	4.000000e-09
GCST008949_2	High chromosomal aberration frequency (chromosome type) in genotoxic compound exposure	8.000000e-06
GCST008954_2	High chromosomal aberration frequency (chromosome type)	5.000000e-06
GCST010307_14	Urinary albumin excretion	8.000000e-09
GCST010866_75	Coronary artery disease	1.000000e-10
GCST011365_152	Myocardial infarction	6.000000e-06

EFO canonical traits (4, from GWAS)

EFO ID	Trait name
EFO:0005035	hippocampal volume
EFO:0005763	pulse pressure measurement
EFO:0009861	chromosome-type aberration frequency
EFO:0004285	albuminuria

MeSH disease descriptors (1)

Descriptor	Name	Tree numbers
C563524	NF1 Microdeletion Syndrome (supp.)

Drugs & pharmacology

Drug and pharmacology data

Is drug target: no

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

CTD chemical–gene interactions

22 total (human), top 22 by PubMed support.

Chemical	Actions (top 5)	PubMed papers
sodium arsenite	increases expression, affects methylation, decreases expression	3
Air Pollutants	decreases expression, increases abundance, increases expression	2
FR900359	increases phosphorylation	1
triphenyl phosphate	affects expression	1
arsenite	affects binding, increases reaction	1
di-n-butylphosphoric acid	affects expression	1
nutlin 3	affects cotreatment, increases secretion	1
Temozolomide	decreases expression	1
Benzo(a)pyrene	decreases methylation	1
Calcitriol	increases expression, affects cotreatment	1
Dactinomycin	affects cotreatment, increases secretion	1
Dichlorodiphenyl Dichloroethylene	increases expression	1
Ivermectin	decreases expression	1
Lead	affects expression	1
Smoke	increases expression, increases abundance	1
Testosterone	affects cotreatment, increases expression, decreases expression	1
Tobacco Smoke Pollution	decreases expression	1
Tretinoin	decreases expression	1
Valproic Acid	affects expression	1
Antirheumatic Agents	increases expression	1
Copper Sulfate	decreases expression	1
Particulate Matter	decreases expression, increases abundance	1

Clinical trials (associated diseases)

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