Protein: Q9UHD9

UniprotKB AC UniprotKB ID Gene name Full name Species Curated set
Q9UHD9 (Uniprot) UBQL2_HUMAN UBQLN2 Ubiquilin-2 human No
Uniprot: Plays an important role in the regulation of different protein degradation mechanisms and pathways including ubiquitin-proteasome system (UPS), autophagy and the endoplasmic reticulum-associated protein degradation (ERAD) pathway. Mediates the proteasomal targeting of misfolded or accumulated proteins for degradation by binding (via UBA domain) to their polyubiquitin chains and by interacting (via ubiquitin-like domain) with the subunits of the proteasome (PubMed:10983987). Plays a role in the ERAD pathway via its interaction with ER-localized proteins FAF2/UBXD8 and HERPUD1 and may form a link between the polyubiquitinated ERAD substrates and the proteasome (PubMed:24215460, PubMed:18307982). Involved in the regulation of macroautophagy and autophagosome formation; required for maturation of autophagy-related protein LC3 from the cytosolic form LC3-I to the membrane-bound form LC3-II and may assist in the maturation of autophagosomes to autolysosomes by mediating autophagosome-lysosome fusion (PubMed:19148225, PubMed:20529957). Negatively regulates the endocytosis of GPCR receptors: AVPR2 and ADRB2, by specifically reducing the rate at which receptor-arrestin complexes concentrate in clathrin-coated pits (CCPs) (PubMed:18199683). more..
MoonDB Dissimilar Functions
GO ID 1 Function 1 Module ID 1 GO ID 2 Function 2 Module ID 2 Association Probability (PrOnto) Interaction Probability (PrOnto)
GO:0019538 protein metabolic process 56, 134, 197, 204, 379, 414, 578, 594, 643 GO:0016070 RNA metabolic process 715 5.15e-08 2.82e-13
GO:0036211 protein modification process 199, 258, 358, 362 GO:0016070 RNA metabolic process 715 5.81e-04 8.44e-05
Biological Processes
Module ID (MoonGO) GO ID (BP) GO Name
22 GO:0019538 protein metabolic process
22 GO:0007165 signal transduction
54 GO:0010467 gene expression
54 GO:0006139 nucleobase-containing compound metabolic process
54 GO:0060255 regulation of macromolecule metabolic process
56 GO:0044267 cellular protein metabolic process
56 GO:0060255 regulation of macromolecule metabolic process
64 GO:0016070 RNA metabolic process
64 GO:0010467 gene expression
64 GO:0060255 regulation of macromolecule metabolic process
74 GO:0060255 regulation of macromolecule metabolic process
87 GO:0034645 cellular macromolecule biosynthetic process
87 GO:0060255 regulation of macromolecule metabolic process
107 GO:0016070 RNA metabolic process
107 GO:0044267 cellular protein metabolic process
107 GO:0034654 nucleobase-containing compound biosynthetic process
107 GO:2000112 regulation of cellular macromolecule biosynthetic process
107 GO:0010468 regulation of gene expression
107 GO:0019219 regulation of nucleobase-containing compound metabolic process
107 GO:0007165 signal transduction
134 GO:0044267 cellular protein metabolic process
134 GO:0060255 regulation of macromolecule metabolic process
134 GO:0007165 signal transduction
145 GO:0060255 regulation of macromolecule metabolic process
178 GO:0060255 regulation of macromolecule metabolic process
178 GO:0007165 signal transduction
197 GO:0044267 cellular protein metabolic process
197 GO:0060255 regulation of macromolecule metabolic process
199 GO:0006464 cellular protein modification process
199 GO:0060255 regulation of macromolecule metabolic process
199 GO:0007165 signal transduction
204 GO:0044267 cellular protein metabolic process
249 GO:0060255 regulation of macromolecule metabolic process
258 GO:0006464 cellular protein modification process
258 GO:0031325 positive regulation of cellular metabolic process
258 GO:0060255 regulation of macromolecule metabolic process
266 GO:0034645 cellular macromolecule biosynthetic process
266 GO:0044271 cellular nitrogen compound biosynthetic process
266 GO:0006464 cellular protein modification process
266 GO:0070887 cellular response to chemical stimulus
266 GO:0032270 positive regulation of cellular protein metabolic process
266 GO:0048584 positive regulation of response to stimulus
266 GO:0006508 proteolysis
266 GO:0050790 regulation of catalytic activity
266 GO:0031326 regulation of cellular biosynthetic process
266 GO:0010468 regulation of gene expression
266 GO:2000026 regulation of multicellular organismal development
266 GO:0019219 regulation of nucleobase-containing compound metabolic process
266 GO:0009966 regulation of signal transduction
343 GO:0060255 regulation of macromolecule metabolic process
358 GO:0006464 cellular protein modification process
358 GO:0060255 regulation of macromolecule metabolic process
358 GO:0007165 signal transduction
360 GO:0060255 regulation of macromolecule metabolic process
362 GO:0006464 cellular protein modification process
362 GO:0060255 regulation of macromolecule metabolic process
372 GO:0019538 protein metabolic process
372 GO:0010468 regulation of gene expression
372 GO:0006351 transcription, DNA-templated
379 GO:0044267 cellular protein metabolic process
379 GO:0009059 macromolecule biosynthetic process
379 GO:0051246 regulation of protein metabolic process
414 GO:0044267 cellular protein metabolic process
414 GO:0006508 proteolysis
414 GO:0060255 regulation of macromolecule metabolic process
414 GO:0007165 signal transduction
446 GO:0007165 signal transduction
455 GO:0010468 regulation of gene expression
528 GO:0007165 signal transduction
531 GO:0019538 protein metabolic process
537 GO:0060255 regulation of macromolecule metabolic process
537 GO:0007165 signal transduction
553 GO:0019538 protein metabolic process
578 GO:0044267 cellular protein metabolic process
578 GO:0031325 positive regulation of cellular metabolic process
578 GO:0010604 positive regulation of macromolecule metabolic process
578 GO:0051173 positive regulation of nitrogen compound metabolic process
578 GO:0048584 positive regulation of response to stimulus
578 GO:0009966 regulation of signal transduction
578 GO:0006355 regulation of transcription, DNA-templated
594 GO:0044267 cellular protein metabolic process
594 GO:0010467 gene expression
594 GO:0060255 regulation of macromolecule metabolic process
616 GO:0090304 nucleic acid metabolic process
643 GO:0044267 cellular protein metabolic process
676 GO:0016070 RNA metabolic process
676 GO:0044267 cellular protein metabolic process
676 GO:0010467 gene expression
676 GO:0060255 regulation of macromolecule metabolic process
700 GO:0019538 protein metabolic process
700 GO:0060255 regulation of macromolecule metabolic process
715 GO:0006396 RNA processing
715 GO:0060255 regulation of macromolecule metabolic process
727 GO:0060255 regulation of macromolecule metabolic process
728 GO:0044267 cellular protein metabolic process
728 GO:0031325 positive regulation of cellular metabolic process
728 GO:0010604 positive regulation of macromolecule metabolic process
728 GO:0051173 positive regulation of nitrogen compound metabolic process
728 GO:0051252 regulation of RNA metabolic process
728 GO:0010468 regulation of gene expression
728 GO:0007165 signal transduction
736 GO:0006139 nucleobase-containing compound metabolic process
736 GO:0060255 regulation of macromolecule metabolic process
753 GO:0044267 cellular protein metabolic process
753 GO:0034654 nucleobase-containing compound biosynthetic process
753 GO:0031325 positive regulation of cellular metabolic process
753 GO:0010604 positive regulation of macromolecule metabolic process
753 GO:0051173 positive regulation of nitrogen compound metabolic process
753 GO:0031326 regulation of cellular biosynthetic process
753 GO:0019219 regulation of nucleobase-containing compound metabolic process
753 GO:0009966 regulation of signal transduction
758 GO:0016070 RNA metabolic process
758 GO:0034645 cellular macromolecule biosynthetic process
758 GO:0044271 cellular nitrogen compound biosynthetic process
758 GO:0044267 cellular protein metabolic process
758 GO:0009893 positive regulation of metabolic process
758 GO:0010468 regulation of gene expression
758 GO:0019219 regulation of nucleobase-containing compound metabolic process
763 GO:0060255 regulation of macromolecule metabolic process
785 GO:0016070 RNA metabolic process
785 GO:0006464 cellular protein modification process
785 GO:0010467 gene expression
785 GO:0060255 regulation of macromolecule metabolic process
796 GO:0034645 cellular macromolecule biosynthetic process
796 GO:0060255 regulation of macromolecule metabolic process
829 GO:0007165 signal transduction
Cellular Components
Module ID (MoonGO) GO ID (CC) GO Name
14 GO:0031410 cytoplasmic vesicle
14 GO:0005829 cytosol
14 GO:0005783 endoplasmic reticulum
22 GO:0005576 extracellular region
22 GO:0005886 plasma membrane
22 GO:0031982 vesicle
54 GO:0070013 intracellular organelle lumen
54 GO:0005634 nucleus
56 GO:0005829 cytosol
60 GO:0005634 nucleus
64 GO:0005634 nucleus
74 GO:0005634 nucleus
87 GO:0005634 nucleus
91 GO:0005576 extracellular region
107 GO:0005829 cytosol
107 GO:0005654 nucleoplasm
119 GO:0005829 cytosol
134 GO:0005829 cytosol
134 GO:0005634 nucleus
143 GO:0005634 nucleus
145 GO:0070013 intracellular organelle lumen
145 GO:0044428 nuclear part
176 GO:0016021 integral component of membrane
176 GO:0031090 organelle membrane
178 GO:0005634 nucleus
199 GO:0012505 endomembrane system
199 GO:0005576 extracellular region
199 GO:0070013 intracellular organelle lumen
204 GO:0005634 nucleus
249 GO:0005829 cytosol
249 GO:0005634 nucleus
249 GO:0031982 vesicle
266 GO:0031410 cytoplasmic vesicle
266 GO:0012505 endomembrane system
266 GO:0005576 extracellular region
266 GO:0070013 intracellular organelle lumen
266 GO:0005634 nucleus
266 GO:0005886 plasma membrane
343 GO:0005634 nucleus
358 GO:0012505 endomembrane system
358 GO:0005615 extracellular space
358 GO:0005886 plasma membrane
358 GO:0031982 vesicle
362 GO:0070013 intracellular organelle lumen
362 GO:0005634 nucleus
368 GO:0005829 cytosol
368 GO:0005634 nucleus
368 GO:0031982 vesicle
372 GO:0005634 nucleus
379 GO:0005829 cytosol
379 GO:0005576 extracellular region
379 GO:0005634 nucleus
396 GO:0012505 endomembrane system
414 GO:0012505 endomembrane system
414 GO:0005615 extracellular space
414 GO:0070013 intracellular organelle lumen
414 GO:0031982 vesicle
438 GO:0005634 nucleus
455 GO:0005634 nucleus
537 GO:0012505 endomembrane system
578 GO:0005634 nucleus
594 GO:0070013 intracellular organelle lumen
594 GO:0005634 nucleus
616 GO:0005654 nucleoplasm
643 GO:0005634 nucleus
676 GO:0070013 intracellular organelle lumen
676 GO:0044428 nuclear part
700 GO:0070013 intracellular organelle lumen
700 GO:0005634 nucleus
700 GO:0031982 vesicle
709 GO:0044421 extracellular region part
715 GO:0031981 nuclear lumen
728 GO:0005829 cytosol
728 GO:0005634 nucleus
736 GO:0005829 cytosol
736 GO:0070013 intracellular organelle lumen
736 GO:0044428 nuclear part
753 GO:0005634 nucleus
758 GO:0005654 nucleoplasm
763 GO:0005634 nucleus
785 GO:0005634 nucleus
796 GO:0005634 nucleus
822 GO:0005634 nucleus
829 GO:0005829 cytosol
829 GO:0031982 vesicle
GO ID (BP) GO Name Evidence Code (GO EC)
GO:0006914 autophagy IEA
GO:0016241 regulation of macroautophagy IMP
GO:0030433 ubiquitin-dependent ERAD pathway IMP
GO:1900186 negative regulation of clathrin-dependent endocytosis IMP
GO:1903071 positive regulation of ER-associated ubiquitin-dependent protein catabolic process IMP
GO:1904021 negative regulation of G-protein coupled receptor internalization IMP
GO:2000785 regulation of autophagosome assembly IMP
GO ID (CC) GO Name Evidence Code (GO EC)
GO:0005634 nucleus IEA
GO:0005737 cytoplasm IDA
GO:0005776 autophagosome IEA
GO:0005829 cytosol TAS
GO:0005886 plasma membrane IDA
GO:0031410 cytoplasmic vesicle IEA
GO ID 1 Component 1 GO ID 2 Component 2 Association Probability (PrOnto) Interaction Probability (PrOnto)
GO:0005634 nucleus GO:0005886 plasma membrane 5.35e-300 7.52e-165
GO:0005634 nucleus GO:0031410 cytoplasmic vesicle 9.49e-36 4.91e-56
Interactor Also a MoonDB EMF protein
G2XKQ0_HUMAN No
DAZP2_HUMAN Yes: Q15038 (MoonDB)
ASCL1_HUMAN No
CTG1B_HUMAN No
A0A024R8L2_HUMAN No
ABI2_HUMAN Yes: Q9NYB9 (MoonDB)
5NT3A_HUMAN No
7B2_HUMAN No
ADRM1_HUMAN No
AGR2_HUMAN No
AGR3_HUMAN No
AMBN_HUMAN No
ANF_HUMAN No
ANR33_HUMAN No
ARAS1_HUMAN No
ARRD3_HUMAN No
ATS3_HUMAN No
ATX7_HUMAN No
AUGN_HUMAN No
B2L11_HUMAN No
BACH_HUMAN No
BAG6_HUMAN No
BAGE4_HUMAN No
BPIA1_HUMAN No
C1QA_HUMAN No
C1QB_HUMAN No
C1QC_HUMAN No
C1QL4_HUMAN No
C1QR1_HUMAN No
C1QT2_HUMAN No
C1QT4_HUMAN No
C99L2_HUMAN No
CA094_HUMAN No
CAD15_HUMAN No
CAD17_HUMAN No
CAN15_HUMAN No
CASA1_HUMAN No
CASB_HUMAN No
CASK_HUMAN No
CCKN_HUMAN No
CCL16_HUMAN No
CCL3_HUMAN No
CCL7_HUMAN No
CD026_HUMAN No
CDIP1_HUMAN No
CDSN_HUMAN No
CF015_HUMAN No
CF218_HUMAN No
CLC11_HUMAN No
CNEP1_HUMAN No
CNNM3_HUMAN No
CO1A2_HUMAN No
CO8A1_HUMAN No
CO8G_HUMAN No
CO9A2_HUMAN No
COAA1_HUMAN No
COHA1_HUMAN No
COLL2_HUMAN No
CSTF2_HUMAN No
CSTFT_HUMAN No
CYR61_HUMAN No
CYTN_HUMAN No
CYTS_HUMAN No
DB115_HUMAN No
DDR1_HUMAN No
DEF1_HUMAN No
DEF6_HUMAN No
DESI1_HUMAN No
DHB12_HUMAN No
DMKN_HUMAN No
DPOE2_HUMAN No
DUS21_HUMAN No
ECM1_HUMAN No
EGFL6_HUMAN No
EKI1_HUMAN No
ERP29_HUMAN No
F168A_HUMAN No
F222B_HUMAN No
FA10_HUMAN No
FA46B_HUMAN No
FBLN3_HUMAN No
FDSCP_HUMAN No
FGF17_HUMAN No
FKBP2_HUMAN No
FND11_HUMAN No
FUCO_HUMAN No
FZD7_HUMAN No
GALA_HUMAN No
GALP_HUMAN No
GHRL_HUMAN No
GP162_HUMAN No
GPX7_HUMAN No
GT252_HUMAN No
GUC2A_HUMAN No
GUC2B_HUMAN No
HEMK1_HUMAN No
HERC3_HUMAN No
HGS_HUMAN No
HNRPU_HUMAN No
HRSL2_HUMAN No
HSP13_HUMAN No
IBP6_HUMAN No
ICAM1_HUMAN No
IGLL1_HUMAN No
IL11_HUMAN No
INSR2_HUMAN No
IP3KB_HUMAN No
IPIL1_HUMAN No
IQCF3_HUMAN No
IST1_HUMAN No
K2C6A_HUMAN No
KLH11_HUMAN No
KLH42_HUMAN No
KR121_HUMAN No
KR193_HUMAN No
LAIR2_HUMAN No
LASP1_HUMAN No
LCN1_HUMAN No
LEUK_HUMAN No
LITAF_HUMAN No
LMO4_HUMAN No
MAGD1_HUMAN No
MBL2_HUMAN No
MID49_HUMAN No
MID51_HUMAN No
MINP1_HUMAN No
MOAP1_HUMAN No
MOT4_HUMAN No
MTR1B_HUMAN No
MTX2_HUMAN No
MYDGF_HUMAN No
NBL1_HUMAN No
NDK3_HUMAN No
NDOR1_HUMAN No
NGAL_HUMAN Yes: P80188 (MoonDB)
NNRD_HUMAN No
NOC4L_HUMAN No
NOL3_HUMAN No
NPBW1_HUMAN No
NPVF_HUMAN No
NRN1L_HUMAN No
NUP58_HUMAN No
OSMR_HUMAN No
P121A_HUMAN No
P3IP1_HUMAN No
PA216_HUMAN No
PAI1_HUMAN No
PARM1_HUMAN No
PCDG9_HUMAN No
PIANP_HUMAN No
PIN1_HUMAN No
PKHB2_HUMAN No
PNMA3_HUMAN No
PODXL_HUMAN No
PORIM_HUMAN No
PPIB_HUMAN No
PPIC_HUMAN No
PPIH_HUMAN No
PR40A_HUMAN Yes: O75400 (MoonDB)
PRAP1_HUMAN No
PROL4_HUMAN No
PS1C2_HUMAN No
PSMD2_HUMAN Yes: Q13200 (MoonDB)
PTGDS_HUMAN No
Q49AM7_HUMAN No
Q8NEJ1_HUMAN No
Q96FB2_HUMAN No
Q96MR4_HUMAN No
QPCT_HUMAN No
RBM24_HUMAN No
RD23A_HUMAN No
RD23B_HUMAN No
RFOX2_HUMAN No
RL40_HUMAN No
RN128_HUMAN No
RN208_HUMAN No
RNF11_HUMAN No
ROA1_HUMAN No
ROA3_HUMAN No
RS27A_HUMAN No
RSMB_HUMAN No
RSRC2_HUMAN No
RTL8A_HUMAN No
RTL8B_HUMAN No
RTL8C_HUMAN No
SC2B2_HUMAN No
SE6L1_HUMAN No
SEMG1_HUMAN No
SEZ6_HUMAN No
SFPA2_HUMAN No
SG11B_HUMAN No
SGTA_HUMAN No
SI11A_HUMAN No
SLIK1_HUMAN No
SLPI_HUMAN No
SMIM2_HUMAN No
SMR3B_HUMAN No
SODE_HUMAN No
SPI2_HUMAN No
SPIT1_HUMAN No
SRGN_HUMAN No
SRP68_HUMAN No
STAM2_HUMAN No
SUOX_HUMAN No
SUSD4_HUMAN No
TFF3_HUMAN No
TIM21_HUMAN No
TIMP2_HUMAN No
TNR18_HUMAN No
TO20L_HUMAN No
TRAF4_HUMAN No
TRI32_HUMAN No
TXD12_HUMAN No
UACA_HUMAN No
UB2V1_HUMAN Yes: Q13404 (MoonDB)
UBAC1_HUMAN No
UBB_HUMAN Yes: P0CG47 (MoonDB)
UBC_HUMAN Yes: P0CG48 (MoonDB)
UBE3A_HUMAN Yes: Q05086 (MoonDB)
UBQL1_HUMAN No
UBQL4_HUMAN No
UBXN1_HUMAN No
UBXN7_HUMAN No
UFSP1_HUMAN No
VENTX_HUMAN No
VIP_HUMAN No
VTNC_HUMAN No
VWC2_HUMAN No
WF10B_HUMAN No
WFD12_HUMAN No
YT011_HUMAN No
ZA2G_HUMAN No
ZFN2A_HUMAN No
ZFN2B_HUMAN No
ZG16B_HUMAN No
ZG16_HUMAN No
ZN205_HUMAN No
PMID Article Title
9853615 Selection system for genes encoding nuclear-targeted proteins.
10675567 A family of ubiquitin-like proteins binds the ATPase domain of Hsp70-like Stch.
10983987 The hPLIC proteins may provide a link between the ubiquitination machinery and the proteasome.
11827521 Structural studies of the interaction between ubiquitin family proteins and proteasome subunit S5a.
15489334 The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).
15772651 The DNA sequence of the human X chromosome.
16813565 Dimerization of ubiquilin is dependent upon the central region of the protein: evidence that the monomer, but not the dimer, is involved in binding presenilins.
17098253 Ubiquitin receptor proteins hHR23a and hPLIC2 interact.
17974005 The full-ORF clone resource of the German cDNA consortium.
18199683 The ubiquitin-like protein PLIC-2 is a negative regulator of G protein-coupled receptor endocytosis.
18307982 Herp enhances ER-associated protein degradation by recruiting ubiquilins.
19148225 PLIC proteins or ubiquilins regulate autophagy-dependent cell survival during nutrient starvation.
19413330 Lys-N and trypsin cover complementary parts of the phosphoproteome in a refined SCX-based approach.
20529957 Ubiquilin functions in autophagy and is degraded by chaperone-mediated autophagy.
20729634 Ubiquilin at a crossroads in protein degradation pathways.
21269460 Initial characterization of the human central proteome.
21857683 Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia.
22560112 UBQLN2 mutations are rare in French and French-Canadian amyotrophic lateral sclerosis.
22628307 Ubiquilins in the crosstalk among proteolytic pathways.
22717235 UBQLN2/ubiquilin 2 mutation and pathology in familial amyotrophic lateral sclerosis.
22814378 N-terminal acetylome analyses and functional insights of the N-terminal acetyltransferase NatB.
22892309 Screening in ALS and FTD patients reveals 3 novel UBQLN2 mutations outside the PXX domain and a pure FTD phenotype.
23459205 Ubiquilin4 is an adaptor protein that recruits Ubiquilin1 to the autophagy machinery.
23541532 Ubiquilin-2 (UBQLN2) binds with high affinity to the C-terminal region of TDP-43 and modulates TDP-43 levels in H4 cells: characterization of inhibition by nucleic acids and 4-aminoquinolines.
24215460 Pathogenic mutation of UBQLN2 impairs its interaction with UBXD8 and disrupts endoplasmic reticulum-associated protein degradation.
24549040 C9ORF72, implicated in amytrophic lateral sclerosis and frontotemporal dementia, regulates endosomal trafficking.
24589709 Ubiquilin 2: a component of the ubiquitin-proteasome system with an emerging role in neurodegeneration.
24674348 The ubiquilin gene family: evolutionary patterns and functional insights.
25616961 ALS-linked mutations in ubiquilin-2 or hnRNPA1 reduce interaction between ubiquilin-2 and hnRNPA1.
OMIM ID Disease Name
300857 Amyotrophic lateral sclerosis 15, with or without frontotemporal dementia
Domain Name Domain ID Source
Ubiquitin_dom IPR000626 InterPro
STI1_HS-bd IPR006636 InterPro
UBA-like_sf IPR009060 InterPro
Ubiquilin IPR015496 InterPro
UBA IPR015940 InterPro
ARM-type_fold IPR016024 InterPro
Ubiquilin-2 IPR028430 InterPro
Ubiquitin-like_domsf IPR029071 InterPro
ubiquitin PF00240 Pfam
UBA PF00627 Pfam
UBA PS50030 PROSITE
UBIQUITIN_2 PS50053 PROSITE
PTHR10677 PTHR10677 PANTHER
PTHR10677:SF5 PTHR10677:SF5 PANTHER
UBA SM00165 SMART
UBQ SM00213 SMART
STI1 SM00727 SMART
SSF46934 SSF46934 SUPFAM
SSF48371 SSF48371 SUPFAM
SSF54236 SSF54236 SUPFAM