DNJB2_HUMAN
Evidence for PS localization
Functions
Functions as a co-chaperone, regulating the substrate binding and activating the ATPase activity of chaperones of the HSP70/heat shock protein 70 family (PubMed, PubMed). In parallel, also contributes to the ubiquitin-dependent proteasomal degradation of misfolded proteins (PubMed, PubMed). Thereby, may regulate the aggregation and promote the functional recovery of misfolded proteins like HTT, MC4R, PRKN, RHO and SOD1 and be crucial for many biological processes (PubMed, PubMed, PubMed, PubMed, PubMed). Isoform 1 which is localized to the endoplasmic reticulum membranes may specifically function in ER-associated protein degradation of misfolded proteins (PubMed). [View more on UniProt]
Protein features
Binary interactions with known binding regions
Interacting region for partners were not defined.
Network
Isoforms
Disease-causing germline mutations
No data found.
Linear motifs
| ELM instance | Name | Type | Start | End | Partner |
|---|---|---|---|---|---|
| ELMI003835 | MOD_CK2_1 | MOD | 247 | 253 | - |
Fingerprint
Molecular function
| Term | Name | % | Distance from the top of the tree | Annotated in DNJB2_HUMAN |
|---|---|---|---|---|
| GO:0017076 | purine nucleotide binding | 50.0000 | 4 | no |
| GO:0030554 | adenyl nucleotide binding | 45.2381 | 5 | no |
| GO:0032555 | purine ribonucleotide binding | 45.2381 | 4 | no |
| GO:0035639 | purine ribonucleoside triphosphate binding | 45.2381 | 4 | no |
| GO:0005524 | ATP binding | 40.4762 | 5 | no |
| GO:0032559 | adenyl ribonucleotide binding | 40.4762 | 5 | no |
| GO:0031625 | ubiquitin protein ligase binding | 35.7143 | 5 | yes |
| GO:0044389 | ubiquitin-like protein ligase binding | 35.7143 | 4 | yes |
| GO:0003723 | RNA binding | 28.5714 | 4 | no |
| GO:0016462 | pyrophosphatase activity | 28.5714 | 5 | no |
| GO:0016818 | hydrolase activity, acting on acid anhydrides, in phosphorus-containing anhydrides | 28.5714 | 4 | no |
| GO:0017111 | ribonucleoside triphosphate phosphatase activity | 28.5714 | 6 | no |
| GO:0016887 | ATP hydrolysis activity | 23.8095 | 7 | no |
| GO:0030544 | Hsp70 protein binding | 4.7619 | 4 | yes |
| GO:0043130 | ubiquitin binding | 4.7619 | 4 | yes |
Biological process
| Term | Name | % | Distance from top the of the tree | Annotated in DNJB2_HUMAN |
|---|---|---|---|---|
| GO:0060255 | regulation of macromolecule metabolic process | 59.5238 | 4 | yes |
| GO:0051171 | regulation of nitrogen compound metabolic process | 54.7619 | 4 | yes |
| GO:0080090 | regulation of primary metabolic process | 54.7619 | 4 | yes |
| GO:0031323 | regulation of cellular metabolic process | 52.3810 | 4 | yes |
| GO:0048522 | positive regulation of cellular process | 50.0000 | 4 | yes |
| GO:0051246 | regulation of protein metabolic process | 45.2381 | 5 | yes |
| GO:0009893 | positive regulation of metabolic process | 42.8571 | 4 | yes |
| GO:0048523 | negative regulation of cellular process | 42.8571 | 4 | yes |
| GO:0010468 | regulation of gene expression | 40.4762 | 5 | no |
| GO:0006996 | organelle organization | 40.4762 | 4 | no |
| GO:0009966 | regulation of signal transduction | 40.4762 | 4 | no |
| GO:0010646 | regulation of cell communication | 40.4762 | 4 | no |
| GO:0010604 | positive regulation of macromolecule metabolic process | 38.0952 | 5 | yes |
| GO:0031399 | regulation of protein modification process | 38.0952 | 6 | yes |
| GO:0051128 | regulation of cellular component organization | 38.0952 | 4 | yes |
| GO:0071310 | cellular response to organic substance | 35.7143 | 4 | no |
| GO:0051173 | positive regulation of nitrogen compound metabolic process | 35.7143 | 5 | yes |
| GO:0009889 | regulation of biosynthetic process | 33.3333 | 4 | no |
| GO:0019219 | regulation of nucleobase-containing compound metabolic process | 33.3333 | 5 | no |
| GO:0031326 | regulation of cellular biosynthetic process | 33.3333 | 5 | no |
| GO:0031325 | positive regulation of cellular metabolic process | 33.3333 | 5 | yes |
| GO:1902531 | regulation of intracellular signal transduction | 30.9524 | 5 | no |
| GO:0010556 | regulation of macromolecule biosynthetic process | 30.9524 | 5 | no |
| GO:0009892 | negative regulation of metabolic process | 30.9524 | 4 | yes |
| GO:0010605 | negative regulation of macromolecule metabolic process | 30.9524 | 5 | yes |
| GO:0051247 | positive regulation of protein metabolic process | 30.9524 | 6 | yes |
| GO:0010941 | regulation of cell death | 30.9524 | 4 | no |
| GO:0042981 | regulation of apoptotic process | 30.9524 | 6 | no |
| GO:0043067 | regulation of programmed cell death | 30.9524 | 5 | no |
| GO:0006355 | regulation of DNA-templated transcription | 28.5714 | 6 | no |
| GO:0051252 | regulation of RNA metabolic process | 28.5714 | 5 | no |
| GO:1903506 | regulation of nucleic acid-templated transcription | 28.5714 | 7 | no |
| GO:2001141 | regulation of RNA biosynthetic process | 28.5714 | 6 | no |
| GO:0036211 | protein modification process | 28.5714 | 4 | no |
| GO:0043412 | macromolecule modification | 28.5714 | 4 | no |
| GO:0048584 | positive regulation of response to stimulus | 28.5714 | 4 | no |
| GO:0009057 | macromolecule catabolic process | 28.5714 | 4 | yes |
| GO:0044087 | regulation of cellular component biogenesis | 28.5714 | 4 | yes |
| GO:0051172 | negative regulation of nitrogen compound metabolic process | 28.5714 | 5 | yes |
| GO:0060548 | negative regulation of cell death | 26.1905 | 5 | no |
| GO:0006986 | response to unfolded protein | 26.1905 | 4 | yes |
| GO:0009894 | regulation of catabolic process | 26.1905 | 4 | yes |
| GO:0030162 | regulation of proteolysis | 26.1905 | 6 | yes |
| GO:0044265 | cellular macromolecule catabolic process | 26.1905 | 4 | yes |
| GO:0051248 | negative regulation of protein metabolic process | 26.1905 | 6 | yes |
| GO:0043066 | negative regulation of apoptotic process | 26.1905 | 7 | no |
| GO:0043069 | negative regulation of programmed cell death | 26.1905 | 6 | no |
| GO:0016192 | vesicle-mediated transport | 23.8095 | 4 | no |
| GO:0009967 | positive regulation of signal transduction | 23.8095 | 5 | no |
| GO:0010647 | positive regulation of cell communication | 23.8095 | 5 | no |
| GO:0023056 | positive regulation of signaling | 23.8095 | 4 | no |
| GO:0051049 | regulation of transport | 23.8095 | 4 | no |
| GO:0031396 | regulation of protein ubiquitination | 23.8095 | 8 | yes |
| GO:0042176 | regulation of protein catabolic process | 23.8095 | 5 | yes |
| GO:1903320 | regulation of protein modification by small protein conjugation or removal | 23.8095 | 7 | yes |
| GO:0080134 | regulation of response to stress | 21.4286 | 4 | no |
| GO:0090304 | nucleic acid metabolic process | 21.4286 | 4 | no |
| GO:0051726 | regulation of cell cycle | 21.4286 | 4 | no |
| GO:0006508 | proteolysis | 21.4286 | 4 | yes |
| GO:0019941 | modification-dependent protein catabolic process | 21.4286 | 6 | yes |
| GO:0031401 | positive regulation of protein modification process | 21.4286 | 7 | yes |
| GO:0032880 | regulation of protein localization | 21.4286 | 5 | yes |
| GO:0043632 | modification-dependent macromolecule catabolic process | 21.4286 | 5 | yes |
| GO:0051603 | proteolysis involved in protein catabolic process | 21.4286 | 5 | yes |
| GO:0060341 | regulation of cellular localization | 21.4286 | 4 | yes |
| GO:1903050 | regulation of proteolysis involved in protein catabolic process | 21.4286 | 7 | yes |
| GO:0010648 | negative regulation of cell communication | 21.4286 | 5 | no |
| GO:0023057 | negative regulation of signaling | 21.4286 | 4 | no |
| GO:0031324 | negative regulation of cellular metabolic process | 21.4286 | 5 | no |
| GO:0051338 | regulation of transferase activity | 21.4286 | 4 | no |
| GO:0031329 | regulation of cellular catabolic process | 19.0476 | 5 | yes |
| GO:0031400 | negative regulation of protein modification process | 19.0476 | 7 | yes |
| GO:0051129 | negative regulation of cellular component organization | 19.0476 | 5 | yes |
| GO:0061136 | regulation of proteasomal protein catabolic process | 19.0476 | 6 | yes |
| GO:0010243 | response to organonitrogen compound | 16.6667 | 4 | yes |
| GO:0042127 | regulation of cell population proliferation | 16.6667 | 4 | yes |
| GO:2000058 | regulation of ubiquitin-dependent protein catabolic process | 16.6667 | 6 | yes |
| GO:0006511 | ubiquitin-dependent protein catabolic process | 14.2857 | 7 | yes |
| GO:0009896 | positive regulation of catabolic process | 14.2857 | 5 | yes |
| GO:0032434 | regulation of proteasomal ubiquitin-dependent protein catabolic process | 14.2857 | 7 | yes |
| GO:0045732 | positive regulation of protein catabolic process | 14.2857 | 6 | yes |
| GO:0045861 | negative regulation of proteolysis | 14.2857 | 7 | yes |
| GO:0090083 | regulation of inclusion body assembly | 14.2857 | 5 | yes |
| GO:0001558 | regulation of cell growth | 11.9048 | 4 | yes |
| GO:0008285 | negative regulation of cell population proliferation | 11.9048 | 5 | yes |
| GO:0031398 | positive regulation of protein ubiquitination | 11.9048 | 9 | yes |
| GO:0043393 | regulation of protein binding | 11.9048 | 4 | yes |
| GO:0045862 | positive regulation of proteolysis | 11.9048 | 7 | yes |
| GO:0090084 | negative regulation of inclusion body assembly | 11.9048 | 6 | yes |
| GO:1901800 | positive regulation of proteasomal protein catabolic process | 11.9048 | 7 | yes |
| GO:1903052 | positive regulation of proteolysis involved in protein catabolic process | 11.9048 | 8 | yes |
| GO:1903321 | negative regulation of protein modification by small protein conjugation or removal | 11.9048 | 8 | yes |
| GO:1903322 | positive regulation of protein modification by small protein conjugation or removal | 11.9048 | 8 | yes |
| GO:0030163 | protein catabolic process | 9.5238 | 4 | yes |
| GO:0031331 | positive regulation of cellular catabolic process | 9.5238 | 6 | yes |
| GO:0032091 | negative regulation of protein binding | 9.5238 | 5 | yes |
| GO:0032436 | positive regulation of proteasomal ubiquitin-dependent protein catabolic process | 9.5238 | 8 | yes |
| GO:0051100 | negative regulation of binding | 9.5238 | 4 | yes |
| GO:1901565 | organonitrogen compound catabolic process | 9.5238 | 4 | yes |
| GO:2000060 | positive regulation of ubiquitin-dependent protein catabolic process | 9.5238 | 7 | yes |
| GO:0010498 | proteasomal protein catabolic process | 7.1429 | 5 | yes |
| GO:0030308 | negative regulation of cell growth | 7.1429 | 5 | yes |
| GO:0043161 | proteasome-mediated ubiquitin-dependent protein catabolic process | 7.1429 | 6 | yes |
| GO:0045926 | negative regulation of growth | 7.1429 | 4 | yes |
| GO:0030433 | ubiquitin-dependent ERAD pathway | 4.7619 | 6 | yes |
| GO:0032781 | positive regulation of ATP-dependent activity | 4.7619 | 4 | yes |
| GO:0034976 | response to endoplasmic reticulum stress | 4.7619 | 4 | yes |
| GO:0036503 | ERAD pathway | 4.7619 | 5 | yes |
| GO:0060249 | anatomical structure homeostasis | 4.7619 | 4 | yes |
| GO:1903332 | regulation of protein folding | 4.7619 | 4 | yes |
Disease
| Term | Name | % | Distance from top the of the tree | Annotated in DNJB2_HUMAN |
|---|---|---|---|---|
| DOID:12377 | spinal muscular atrophy | 4.7619 | 6 | no |
| DOID:1289 | neurodegenerative disease | 4.7619 | 4 | no |
| DOID:231 | motor neuron disease | 4.7619 | 5 | no |
| DOID:331 | central nervous system disease | 4.7619 | 3 | no |
All partners
| Uniprot ID | Details | Highest evidence | Localizing into PSD | HPA (protein expression in neurons) |
|---|---|---|---|---|
| UBQL1_HUMAN | [view interactions] | Low throughput | no | yes |
| UBC_HUMAN | [view entry] [view interactions] | Low throughput | yes | yes |
| HSP74_HUMAN | [view entry] [view interactions] | Low throughput | yes | yes |
| ATX3_HUMAN | [view interactions] | Low throughput | no | yes |
| PSA6_HUMAN | [view interactions] | Low throughput | no | yes |
| HSP7C_HUMAN | [view entry] [view interactions] | Low throughput | yes | yes |
| OPSD_HUMAN | [view interactions] | Low throughput | no | no |
| SODC_HUMAN | [view interactions] | Low throughput | no | yes |
| HS90B_HUMAN | [view entry] [view interactions] | Low throughput | yes | yes |
| HS71B_HUMAN | [view entry] [view interactions] | Low throughput | yes | yes |
| HS71A_HUMAN | [view entry] [view interactions] | Low throughput | yes | yes |
| SQSTM_HUMAN | [view interactions] | Low throughput | no | yes |
| SPB5_HUMAN | [view interactions] | Low throughput | no | no |
| TADBP_HUMAN | [view interactions] | Low throughput | no | yes |
| CSK21_HUMAN | [view entry] [view interactions] | Low throughput | yes | yes |
| SMAD4_HUMAN | [view interactions] | High throughput | no | yes |
| HDA11_HUMAN | [view interactions] | High throughput | no | no |
| XPO7_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| SHLD3_HUMAN | [view interactions] | High throughput | no | yes |
| CFTR_HUMAN | [view interactions] | High throughput | no | no |
| PRIO_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| ERBB2_HUMAN | [view entry] [view interactions] | High throughput | yes | no |
| MLF1_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| MLF2_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| PSMD2_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| M3K1_HUMAN | [view interactions] | High throughput | no | yes |
| UB2V1_HUMAN | [view entry] [view interactions] | High throughput | yes | no |
| UBA1_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| UBE2N_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| ERR2_HUMAN | [view interactions] | High throughput | no | no |
| DNJB8_HUMAN | [view interactions] | High throughput | no | no |
| NSE4A_HUMAN | [view interactions] | High throughput | no | yes |
| DNJB6_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| UBB_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| BGAL_HUMAN | [view interactions] | High throughput | no | yes |
| TROP_HUMAN | [view interactions] | High throughput | no | no |
| ATP7A_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| ATP7B_HUMAN | [view interactions] | High throughput | no | no |
| BIG2_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| PPGB_HUMAN | [view interactions] | High throughput | no | yes |
| PJA1_HUMAN | [view interactions] | High throughput | no | yes |
| FA83H_HUMAN | [view interactions] | High throughput | no | yes |
| MO4L1_HUMAN | [view interactions] | High throughput | no | yes |
| GET3_HUMAN | [view interactions] | High throughput | no | yes |
| BIRC2_HUMAN | [view interactions] | High throughput | no | yes |
| RN19B_HUMAN | [view interactions] | High throughput | no | yes |
| CYHR1_HUMAN | [view interactions] | High throughput | no | yes |
| RFWD3_HUMAN | [view interactions] | High throughput | no | yes |
| KCTD3_HUMAN | [view interactions] | High throughput | no | yes |
| TRAF2_HUMAN | [view interactions] | High throughput | no | yes |
| LACC1_HUMAN | [view interactions] | High throughput | no | yes |
| NEK4_HUMAN | [view interactions] | High throughput | no | yes |
| RN115_HUMAN | [view interactions] | High throughput | no | yes |
| RNF12_HUMAN | [view interactions] | High throughput | no | yes |
| CYLD_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| RNF4_HUMAN | [view interactions] | High throughput | no | yes |
| ESR2_HUMAN | [view interactions] | High throughput | no | no |
| LMBRL_HUMAN | [view interactions] | High throughput | no | no |
| ARL1_HUMAN | [view interactions] | High throughput | no | yes |
| HS71L_HUMAN | [view entry] [view interactions] | High throughput | yes | no |
| HSP72_HUMAN | [view entry] [view interactions] | High throughput | yes | no |
| HSP76_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| GRP75_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| RS27A_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| DNJC7_HUMAN | [view interactions] | High throughput | no | yes |
| BAG5_HUMAN | [view entry] [view interactions] | High throughput | yes | no |
| CHIP_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| TM231_HUMAN | [view interactions] | High throughput | no | yes |
| GRPE1_HUMAN | [view interactions] | High throughput | no | yes |
| TBB6_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| FKBP8_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| BAG2_HUMAN | [view entry] [view interactions] | High throughput | yes | no |
| DX39A_HUMAN | [view interactions] | High throughput | no | yes |
| DBLOH_HUMAN | [view interactions] | High throughput | no | no |
| KCNE3_HUMAN | [view interactions] | High throughput | no | yes |
| WRP73_HUMAN | [view interactions] | High throughput | no | no |
| RN185_HUMAN | [view interactions] | High throughput | no | no |
| K0100_HUMAN | [view interactions] | High throughput | no | yes |
| TBA1A_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| GET4_HUMAN | [view interactions] | High throughput | no | yes |
| DHDH_HUMAN | [view interactions] | High throughput | no | no |
| UBP34_HUMAN | [view interactions] | High throughput | no | yes |
| PGAM2_HUMAN | [view entry] [view interactions] | High throughput | yes | no |
| PAAT_HUMAN | [view interactions] | High throughput | no | yes |
| MY18A_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| ADT4_HUMAN | [view entry] [view interactions] | High throughput | yes | no |
| MARH7_HUMAN | [view interactions] | High throughput | no | yes |
| ACTB_HUMAN | [view entry] [view interactions] | High throughput | yes | no |
| SPRTN_HUMAN | [view interactions] | High throughput | no | yes |
| NEDD8_HUMAN | [view interactions] | High throughput | no | yes |
| FBX25_HUMAN | [view interactions] | High throughput | no | no |
| METH_HUMAN | [view interactions] | High throughput | no | yes |
| SRP14_HUMAN | [view interactions] | High throughput | no | no |
| STIP1_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| HS74L_HUMAN | [view entry] [view interactions] | High throughput | yes | no |
| BBS7_HUMAN | [view interactions] | High throughput | no | no |
| SNX6_HUMAN | [view entry] [view interactions] | High throughput | yes | no |
| SEH1_HUMAN | [view interactions] | High throughput | no | no |
| BBS2_HUMAN | [view interactions] | High throughput | no | no |
| RCC1_HUMAN | [view interactions] | High throughput | no | yes |
| GRB2_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| SYTC_HUMAN | [view interactions] | High throughput | no | yes |
| SYNPO_HUMAN | [view entry] [view interactions] | High throughput | yes | no |