UniProtKB - Q6NZY4 (ZCHC8_HUMAN)
Zinc finger CCHC domain-containing protein 8
ZCCHC8
Functioni
Regions
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Zinc fingeri | 227 – 244 | CCHC-typePROSITE-ProRule annotationAdd BLAST | 18 |
GO - Molecular functioni
- RNA binding Source: UniProtKB
- zinc ion binding Source: InterPro
GO - Biological processi
- mRNA splicing, via spliceosome Source: UniProtKB
- ncRNA processing Source: UniProtKB
- snRNA catabolic process Source: UniProtKB
Keywordsi
Biological process | mRNA processing, mRNA splicing |
Ligand | Metal-binding, Zinc |
Enzyme and pathway databases
PathwayCommonsi | Q6NZY4 |
Names & Taxonomyi
Protein namesi | Recommended name: Zinc finger CCHC domain-containing protein 8Alternative name(s): TRAMP-like complex RNA-binding factor ZCCHC8 |
Gene namesi | Name:ZCCHC8 |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:25265, ZCCHC8 |
MIMi | 616381, gene |
neXtProti | NX_Q6NZY4 |
VEuPathDBi | HostDB:ENSG00000033030.13 |
Subcellular locationi
Nucleus
- nucleoplasm 2 Publications
- Nucleus PROSITE-ProRule annotation
Note: Excluded from nucleolus.1 Publication
Nucleus
- catalytic step 2 spliceosome Source: UniProtKB
- nuclear body Source: HPA
- nucleoplasm Source: UniProtKB
- nucleus Source: UniProtKB
- TRAMP complex Source: UniProtKB
Keywords - Cellular componenti
Nucleus, SpliceosomePathology & Biotechi
Involvement in diseasei
Pulmonary fibrosis, and/or bone marrow failure, telomere-related, 5 (PFBMFT5)1 Publication
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_083448 | 186 | P → L in PFBMFT5; decreased levels of mature TERC in patient cells consistent with impaired function in RNA processing; decreased levels of mutant protein in patient cells. 1 Publication | 1 |
Mutagenesis
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Mutagenesisi | 295 | L → E: Impaired interaction with ZCCHC8; when associated with E-299. 1 Publication | 1 | |
Mutagenesisi | 299 | L → E: Impaired interaction with ZCCHC8; when associated with E-295. 1 Publication | 1 | |
Mutagenesisi | 309 | F → A: Reduced interaction with ZCCHC8; when associated with E-313. 1 Publication | 1 | |
Mutagenesisi | 313 | M → E: Reduced interaction with ZCCHC8; when associated with A-309. 1 Publication | 1 | |
Mutagenesisi | 492 | T → A: Impaired phosphorylation by GSK3. 1 Publication | 1 | |
Mutagenesisi | 662 | D → A: Does not alter RNA helicase activity of NEXT complex; when associated with K-666. 1 Publication | 1 | |
Mutagenesisi | 666 | F → K: Does not alter RNA helicase activity of NEXT complex; when associated with A-662. 1 Publication | 1 | |
Mutagenesisi | 673 | F → A: Does not affect RNA helicase activity of NEXT complex; when associated with A-675. 1 Publication | 1 | |
Mutagenesisi | 674 | E → A: Does not affect RNA helicase activity of NEXT complex; when associated with A-676. 1 Publication | 1 | |
Mutagenesisi | 675 | F → A: Does not affect RNA helicase activity of NEXT complex; when associated with A-673. 1 Publication | 1 | |
Mutagenesisi | 676 | E → A: Does not affect RNA helicase activity of NEXT complex; when associated with A-674. 1 Publication | 1 | |
Mutagenesisi | 688 | I → E: Loss of RNA helicase activity of NEXT complex; when associated with E-692. 1 Publication | 1 | |
Mutagenesisi | 692 | L → E: Loss of RNA helicase activity of NEXT complex; when associated with E-688. 1 Publication | 1 |
Keywords - Diseasei
Disease variantOrganism-specific databases
DisGeNETi | 55596 |
MalaCardsi | ZCCHC8 |
MIMi | 618674, phenotype |
OpenTargetsi | ENSG00000033030 |
PharmGKBi | PA134889410 |
Miscellaneous databases
Pharosi | Q6NZY4, Tdark |
Genetic variation databases
BioMutai | ZCCHC8 |
DMDMi | 66774213 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Initiator methioninei | RemovedCombined sources | |||
ChainiPRO_0000150960 | 2 – 707 | Zinc finger CCHC domain-containing protein 8Add BLAST | 706 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Modified residuei | 2 | N-acetylalanineCombined sources | 1 | |
Modified residuei | 342 | PhosphothreonineCombined sources | 1 | |
Cross-linki | 413 | Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in SUMO2)Combined sources | ||
Modified residuei | 472 | PhosphothreonineCombined sources | 1 | |
Modified residuei | 479 | PhosphothreonineCombined sources | 1 | |
Modified residuei | 485 | PhosphothreonineCombined sources | 1 | |
Modified residuei | 492 | Phosphothreonine; by GSK3Combined sources1 Publication | 1 | |
Modified residuei | 577 | PhosphothreonineCombined sources | 1 | |
Modified residuei | 598 | PhosphoserineCombined sources | 1 | |
Modified residuei | 648 | PhosphothreonineCombined sources | 1 | |
Modified residuei | 649 | PhosphoserineCombined sources | 1 | |
Modified residuei | 658 | PhosphoserineCombined sources | 1 | |
Modified residuei | 695 | PhosphoserineCombined sources | 1 |
Post-translational modificationi
Keywords - PTMi
Acetylation, Isopeptide bond, Phosphoprotein, Ubl conjugationProteomic databases
CPTACi | CPTAC-1021 |
EPDi | Q6NZY4 |
jPOSTi | Q6NZY4 |
MassIVEi | Q6NZY4 |
MaxQBi | Q6NZY4 |
PaxDbi | Q6NZY4 |
PeptideAtlasi | Q6NZY4 |
PRIDEi | Q6NZY4 |
ProteomicsDBi | 66798 [Q6NZY4-1] 66799 [Q6NZY4-2] |
PTM databases
iPTMneti | Q6NZY4 |
PhosphoSitePlusi | Q6NZY4 |
Expressioni
Inductioni
Gene expression databases
Bgeei | ENSG00000033030, Expressed in cervix squamous epithelium and 229 other tissues |
ExpressionAtlasi | Q6NZY4, baseline and differential |
Genevisiblei | Q6NZY4, HS |
Organism-specific databases
HPAi | ENSG00000033030, Low tissue specificity |
Interactioni
Subunit structurei
Component of a nuclear TRAMP-like complex, an ATP-dependent exosome regulatory complex consisting of a helicase (MTREX), an oligadenylate polymerase (TENT4B or TENT4A), and a substrate specific RNA-binding factor (ZCCHC7 or ZCCHC8). Several TRAMP-like complexes exist with specific compositions and are associated with nuclear, or nucleolar RNA exosomes. Identified in the spliceosome C complex.
Component of the nuclear exosome targeting (NEXT) complex composed of MTREX, ZCCHC8, and RBM7 that directs a subset of non-coding short-lived RNAs for exosomal degradation (PubMed:27905398, PubMed:27871484).
Interacts with proteins involved in RNA processing and degradation such as MTREX and RBM7; interaction with MTREX enhances MTREX RNA helicase activity and bridges between RBM7 and MTREX (PubMed:16263084, PubMed:27905398, PubMed:27871484).
Interacts with TERC, the telomerase RNA component (PubMed:31488579).
5 PublicationsBinary interactionsi
Q6NZY4
With | #Exp. | IntAct |
---|---|---|
BRCA1 [P38398] | 2 | EBI-1263058,EBI-349905 |
Protein-protein interaction databases
BioGRIDi | 120739, 89 interactors |
CORUMi | Q6NZY4 |
IntActi | Q6NZY4, 47 interactors |
MINTi | Q6NZY4 |
STRINGi | 9606.ENSP00000438993 |
Miscellaneous databases
RNActi | Q6NZY4, protein |
Structurei
Secondary structure
3D structure databases
SMRi | Q6NZY4 |
ModBasei | Search... |
PDBe-KBi | Search... |
Family & Domainsi
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 286 – 299 | RBM7 bindingCombined sources1 PublicationAdd BLAST | 14 | |
Regioni | 309 – 324 | RBM7 bindingCombined sources1 PublicationAdd BLAST | 16 | |
Regioni | 659 – 707 | MTREX bindingCombined sources1 PublicationAdd BLAST | 49 |
Coiled coil
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Coiled coili | 45 – 80 | Sequence analysisAdd BLAST | 36 | |
Coiled coili | 516 – 539 | Sequence analysisAdd BLAST | 24 |
Compositional bias
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Compositional biasi | 466 – 501 | Pro-richPROSITE-ProRule annotationAdd BLAST | 36 |
Domaini
Sequence similaritiesi
Zinc finger
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Zinc fingeri | 227 – 244 | CCHC-typePROSITE-ProRule annotationAdd BLAST | 18 |
Keywords - Domaini
Coiled coil, Zinc-fingerPhylogenomic databases
eggNOGi | KOG2673, Eukaryota |
GeneTreei | ENSGT00390000011475 |
InParanoidi | Q6NZY4 |
OMAi | EDHQMKD |
OrthoDBi | 338817at2759 |
PhylomeDBi | Q6NZY4 |
TreeFami | TF321837 |
Family and domain databases
InterProi | View protein in InterPro IPR006568, PSP_pro-rich IPR001878, Znf_CCHC |
Pfami | View protein in Pfam PF04046, PSP, 1 hit PF00098, zf-CCHC, 1 hit |
SMARTi | View protein in SMART SM00581, PSP, 1 hit SM00343, ZnF_C2HC, 1 hit |
PROSITEi | View protein in PROSITE PS50158, ZF_CCHC, 1 hit |
s (2+)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry describes 2 produced by isoformsialternative splicing. AlignAdd to basketThis entry has 2 described isoforms and 3 potential isoforms that are computationally mapped.Show allAlign All
This isoform has been chosen as the sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry. canonicali
10 20 30 40 50
MAAEVYFGDL ELFEPFDHPE ESIPKPVHTR FKDDDGDEED ENGVGDAELR
60 70 80 90 100
ERLRQCEETI EQLRAENQEL KRKLNILTRP SGILVNDTKL DGPILQILFM
110 120 130 140 150
NNAISKQYHQ EIEEFVSNLV KRFEEQQKND VEKTSFNLLP QPSSIVLEED
160 170 180 190 200
HKVEESCAIK NNKEAFSVVG SVLYFTNFCL DKLGQPLLNE NPQLSEGWEI
210 220 230 240 250
PKYHQVFSHI VSLEGQEIQV KAKRPKPHCF NCGSEEHQMK DCPMPRNAAR
260 270 280 290 300
ISEKRKEYMD ACGEANNQNF QQRYHAEEVE ERFGRFKPGV ISEELQDALG
310 320 330 340 350
VTDKSLPPFI YRMRQLGYPP GWLKEAELEN SGLALYDGKD GTDGETEVGE
360 370 380 390 400
IQQNKSVTYD LSKLVNYPGF NISTPRGIPD EWRIFGSIPM QACQQKDVFA
410 420 430 440 450
NYLTSNFQAP GVKSGNKRSS SHSSPGSPKK QKNESNSAGS PADMELDSDM
460 470 480 490 500
EVPHGSQSSE SFQFQPPLPP DTPPLPRGTP PPVFTPPLPK GTPPLTPSDS
510 520 530 540 550
PQTRTASGAV DEDALTLEEL EEQQRRIWAA LEQAESVNSD SDVPVDTPLT
560 570 580 590 600
GNSVASSPCP NELDLPVPEG KTSEKQTLDE PEVPEIFTKK SEAGHASSPD
610 620 630 640 650
SEVTSLCQKE KAELAPVNTE GALLDNGSVV PNCDISNGGS QKLFPADTSP
660 670 680 690 700
STATKIHSPI PDMSKFATGI TPFEFENMAE STGMYLRIRS LLKNSPRNQQ
KNKKASE
Computationally mapped potential isoform sequencesi
There are 3 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketF5GX80 | F5GX80_HUMAN | Zinc finger CCHC domain-containing ... | ZCCHC8 | 211 | Annotation score: | ||
F5H6J5 | F5H6J5_HUMAN | Zinc finger CCHC domain-containing ... | ZCCHC8 | 185 | Annotation score: | ||
F5GYI9 | F5GYI9_HUMAN | Zinc finger CCHC domain-containing ... | ZCCHC8 | 38 | Annotation score: |
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 20 | E → G in BAB55308 (PubMed:14702039).Curated | 1 | |
Sequence conflicti | 216 – 219 | QEIQ → DAWV in AAH17704 (PubMed:15489334).Curated | 4 | |
Sequence conflicti | 326 | A → V in AAH65918 (PubMed:15489334).Curated | 1 | |
Sequence conflicti | 461 | S → G in BAB55308 (PubMed:14702039).Curated | 1 | |
Sequence conflicti | 582 | E → G in BAC11105 (PubMed:14702039).Curated | 1 | |
Sequence conflicti | 663 | M → V in CAB75658 (PubMed:17974005).Curated | 1 |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_083448 | 186 | P → L in PFBMFT5; decreased levels of mature TERC in patient cells consistent with impaired function in RNA processing; decreased levels of mutant protein in patient cells. 1 Publication | 1 | |
Natural variantiVAR_034585 | 672 | P → A. Corresponds to variant dbSNP:rs1063155Ensembl. | 1 |
Alternative sequence
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Alternative sequenceiVSP_013717 | 1 – 238 | Missing in isoform 2. 1 PublicationAdd BLAST | 238 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AK027702 mRNA Translation: BAB55308.1 AK074638 mRNA Translation: BAC11105.1 AL157433 mRNA Translation: CAB75657.1 AL157434 mRNA Translation: CAB75658.1 BC017704 mRNA Translation: AAH17704.2 BC065918 mRNA Translation: AAH65918.1 |
CCDSi | CCDS86340.1 [Q6NZY4-2] |
PIRi | T46929 T46930 |
RefSeqi | NP_060082.2, NM_017612.4 [Q6NZY4-1] |
Genome annotation databases
Ensembli | ENST00000536306; ENSP00000441423; ENSG00000033030 [Q6NZY4-2] ENST00000543897; ENSP00000438993; ENSG00000033030 [Q6NZY4-2] ENST00000633063; ENSP00000488055; ENSG00000033030 [Q6NZY4-1] ENST00000672018; ENSP00000500728; ENSG00000033030 [Q6NZY4-1] |
GeneIDi | 55596 |
KEGGi | hsa:55596 |
UCSCi | uc009zxp.4, human [Q6NZY4-1] |
Keywords - Coding sequence diversityi
Alternative splicingSimilar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AK027702 mRNA Translation: BAB55308.1 AK074638 mRNA Translation: BAC11105.1 AL157433 mRNA Translation: CAB75657.1 AL157434 mRNA Translation: CAB75658.1 BC017704 mRNA Translation: AAH17704.2 BC065918 mRNA Translation: AAH65918.1 |
CCDSi | CCDS86340.1 [Q6NZY4-2] |
PIRi | T46929 T46930 |
RefSeqi | NP_060082.2, NM_017612.4 [Q6NZY4-1] |
3D structure databases
Select the link destinations: PDBei RCSB PDBi PDBji Links Updated | PDB entry | Method | Resolution (Å) | Chain | Positions | PDBsum |
5LXR | X-ray | 2.00 | B | 285-324 | [»] | |
5LXY | X-ray | 2.85 | C/D/F/H/J/L/N | 285-324 | [»] | |
6C90 | X-ray | 2.20 | B | 659-707 | [»] | |
SMRi | Q6NZY4 | |||||
ModBasei | Search... | |||||
PDBe-KBi | Search... |
Protein-protein interaction databases
BioGRIDi | 120739, 89 interactors |
CORUMi | Q6NZY4 |
IntActi | Q6NZY4, 47 interactors |
MINTi | Q6NZY4 |
STRINGi | 9606.ENSP00000438993 |
PTM databases
iPTMneti | Q6NZY4 |
PhosphoSitePlusi | Q6NZY4 |
Genetic variation databases
BioMutai | ZCCHC8 |
DMDMi | 66774213 |
Proteomic databases
CPTACi | CPTAC-1021 |
EPDi | Q6NZY4 |
jPOSTi | Q6NZY4 |
MassIVEi | Q6NZY4 |
MaxQBi | Q6NZY4 |
PaxDbi | Q6NZY4 |
PeptideAtlasi | Q6NZY4 |
PRIDEi | Q6NZY4 |
ProteomicsDBi | 66798 [Q6NZY4-1] 66799 [Q6NZY4-2] |
Protocols and materials databases
Antibodypediai | 31655, 109 antibodies |
Genome annotation databases
Ensembli | ENST00000536306; ENSP00000441423; ENSG00000033030 [Q6NZY4-2] ENST00000543897; ENSP00000438993; ENSG00000033030 [Q6NZY4-2] ENST00000633063; ENSP00000488055; ENSG00000033030 [Q6NZY4-1] ENST00000672018; ENSP00000500728; ENSG00000033030 [Q6NZY4-1] |
GeneIDi | 55596 |
KEGGi | hsa:55596 |
UCSCi | uc009zxp.4, human [Q6NZY4-1] |
Organism-specific databases
CTDi | 55596 |
DisGeNETi | 55596 |
GeneCardsi | ZCCHC8 |
HGNCi | HGNC:25265, ZCCHC8 |
HPAi | ENSG00000033030, Low tissue specificity |
MalaCardsi | ZCCHC8 |
MIMi | 616381, gene 618674, phenotype |
neXtProti | NX_Q6NZY4 |
OpenTargetsi | ENSG00000033030 |
PharmGKBi | PA134889410 |
VEuPathDBi | HostDB:ENSG00000033030.13 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG2673, Eukaryota |
GeneTreei | ENSGT00390000011475 |
InParanoidi | Q6NZY4 |
OMAi | EDHQMKD |
OrthoDBi | 338817at2759 |
PhylomeDBi | Q6NZY4 |
TreeFami | TF321837 |
Enzyme and pathway databases
PathwayCommonsi | Q6NZY4 |
Miscellaneous databases
BioGRID-ORCSi | 55596, 12 hits in 212 CRISPR screens |
ChiTaRSi | ZCCHC8, human |
GeneWikii | ZCCHC8 |
GenomeRNAii | 55596 |
Pharosi | Q6NZY4, Tdark |
PROi | PR:Q6NZY4 |
RNActi | Q6NZY4, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000033030, Expressed in cervix squamous epithelium and 229 other tissues |
ExpressionAtlasi | Q6NZY4, baseline and differential |
Genevisiblei | Q6NZY4, HS |
Family and domain databases
InterProi | View protein in InterPro IPR006568, PSP_pro-rich IPR001878, Znf_CCHC |
Pfami | View protein in Pfam PF04046, PSP, 1 hit PF00098, zf-CCHC, 1 hit |
SMARTi | View protein in SMART SM00581, PSP, 1 hit SM00343, ZnF_C2HC, 1 hit |
PROSITEi | View protein in PROSITE PS50158, ZF_CCHC, 1 hit |
ProtoNeti | Search... |
MobiDBi | Search... |
Entry informationi
Entry namei | ZCHC8_HUMAN | |
Accessioni | Q6NZY4Primary (citable) accession number: Q6NZY4 Secondary accession number(s): Q7L2P6 Q9NSS3 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | May 10, 2005 |
Last sequence update: | May 10, 2005 | |
Last modified: | April 7, 2021 | |
This is version 164 of the entry and version 2 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any 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. |
Miscellaneousi
Keywords - Technical termi
3D-structure, Reference proteomeDocuments
- Human chromosome 12
Human chromosome 12: entries, gene names and cross-references to MIM - Human entries with genetic variants
List of human entries with genetic variants - Human variants curated from literature reports
Index of human variants curated from literature reports - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - PDB cross-references
Index of Protein Data Bank (PDB) cross-references - SIMILARITY comments
Index of protein domains and families