PCCA_HUMAN
Evidence for PS localization
Functions
This is one of the 2 subunits of the biotin-dependent propionyl-CoA carboxylase (PCC), a mitochondrial enzyme involved in the catabolism of odd chain fatty acids, branched-chain amino acids isoleucine, threonine, methionine, and valine and other metabolites (PubMed, PubMed). Propionyl-CoA carboxylase catalyzes the carboxylation of propionyl-CoA/propanoyl-CoA to D-methylmalonyl-CoA/(S)-methylmalonyl-CoA (PubMed, PubMed, PubMed). Within the holoenzyme, the alpha subunit catalyzes the ATP-dependent carboxylation of the biotin carried by the biotin carboxyl carrier (BCC) domain, while the beta subunit then transfers the carboxyl group from carboxylated biotin to propionyl-CoA . Propionyl-CoA carboxylase also significantly acts on butyryl-CoA/butanoyl-CoA, which is converted to ethylmalonyl-CoA/(2S)-ethylmalonyl-CoA at a much lower rate (PubMed). Other alternative minor substrates include (2E)-butenoyl-CoA/crotonoyl-CoA . [View more on UniProt]
Protein features
Binary interactions with known binding regions
To display all evidence describing the interaction with a partner, click on the protein name left to the bars.
Network
Isoforms
Disease-causing germline mutations
| Position | Amino acid | Mutation | Disease | Overlap with binding region |
|---|---|---|---|---|
| 75 | Ala | Pro | PropionicacidemiatypeI(PA-1) | - |
| 77 | Arg | Trp | PropionicacidemiatypeI(PA-1) | - |
| 138 | Ala | Thr | PropionicacidemiatypeI(PA-1) | - |
| 164 | Ile | Thr | PropionicacidemiatypeI(PA-1) | - |
| 229 | Met | Lys | PropionicacidemiatypeI(PA-1) | - |
| 297 | Gln | Arg | PropionicacidemiatypeI(PA-1) | - |
| 368 | Asp | Gly | PropionicacidemiatypeI(PA-1) | - |
| 373 | Met | Lys | PropionicacidemiatypeI(PA-1) | - |
| 379 | Gly | Val | PropionicacidemiatypeI(PA-1) | - |
| 399 | Arg | Gln | PropionicacidemiatypeI(PA-1) | - |
| 423 | Pro | Leu | PropionicacidemiatypeI(PA-1) | - |
| 559 | Trp | Leu | PropionicacidemiatypeI(PA-1) | - |
| 631 | Gly | Arg | PropionicacidemiatypeI(PA-1) | - |
| 668 | Gly | Arg | PropionicacidemiatypeI(PA-1) | - |
| 197 | Gly | Glu | PropionicacidemiatypeI(PA-1) | - |
| 398 | Cys | Arg | PropionicacidemiatypeI(PA-1) | - |
Linear motifs
No annotated instance was found. To search for linear motifs, use the ELM prediction server.
Fingerprint
Molecular function
| Term | Name | % | Distance from the top of the tree | Annotated in PCCA_HUMAN |
|---|---|---|---|---|
| GO:0000166 | nucleotide binding | 41.9355 | 4 | yes |
| GO:0017076 | purine nucleotide binding | 41.9355 | 5 | yes |
| GO:0032555 | purine ribonucleotide binding | 41.9355 | 4 | yes |
| GO:0035639 | purine ribonucleoside triphosphate binding | 41.9355 | 4 | yes |
| GO:0043168 | anion binding | 41.9355 | 4 | yes |
| GO:0043169 | cation binding | 35.4839 | 4 | yes |
| GO:0046872 | metal ion binding | 35.4839 | 5 | yes |
| GO:0005524 | ATP binding | 29.0323 | 5 | yes |
| GO:0030554 | adenyl nucleotide binding | 29.0323 | 6 | yes |
| GO:0032559 | adenyl ribonucleotide binding | 29.0323 | 5 | yes |
| GO:0003723 | RNA binding | 29.0323 | 4 | no |
| GO:0031625 | ubiquitin protein ligase binding | 25.8065 | 5 | no |
| GO:0044389 | ubiquitin-like protein ligase binding | 25.8065 | 4 | no |
| GO:0016462 | pyrophosphatase activity | 22.5806 | 5 | no |
| GO:0016818 | hydrolase activity, acting on acid anhydrides, in phosphorus-containing anhydrides | 22.5806 | 4 | no |
| GO:0017111 | ribonucleoside triphosphate phosphatase activity | 22.5806 | 6 | no |
| GO:0016421 | CoA carboxylase activity | 12.9032 | 4 | yes |
| GO:0031406 | carboxylic acid binding | 9.6774 | 4 | yes |
| GO:0033293 | monocarboxylic acid binding | 9.6774 | 5 | yes |
Biological process
| Term | Name | % | Distance from top the of the tree | Annotated in PCCA_HUMAN |
|---|---|---|---|---|
| GO:0031323 | regulation of cellular metabolic process | 51.6129 | 4 | no |
| GO:0009889 | regulation of biosynthetic process | 48.3871 | 4 | no |
| GO:0010468 | regulation of gene expression | 48.3871 | 6 | no |
| GO:0010556 | regulation of macromolecule biosynthetic process | 48.3871 | 5 | no |
| GO:0031326 | regulation of cellular biosynthetic process | 48.3871 | 5 | no |
| GO:0060255 | regulation of macromolecule metabolic process | 48.3871 | 4 | no |
| GO:0048522 | positive regulation of cellular process | 45.1613 | 4 | no |
| GO:0048523 | negative regulation of cellular process | 45.1613 | 4 | no |
| GO:0009892 | negative regulation of metabolic process | 41.9355 | 4 | no |
| GO:0010605 | negative regulation of macromolecule metabolic process | 38.7097 | 5 | no |
| GO:0022607 | cellular component assembly | 38.7097 | 4 | no |
| GO:0051128 | regulation of cellular component organization | 35.4839 | 4 | no |
| GO:0080090 | regulation of primary metabolic process | 35.4839 | 4 | no |
| GO:0031324 | negative regulation of cellular metabolic process | 35.4839 | 5 | no |
| GO:0009890 | negative regulation of biosynthetic process | 32.2581 | 5 | no |
| GO:0010558 | negative regulation of macromolecule biosynthetic process | 32.2581 | 6 | no |
| GO:0031327 | negative regulation of cellular biosynthetic process | 32.2581 | 6 | no |
| GO:0006996 | organelle organization | 32.2581 | 4 | no |
| GO:0009893 | positive regulation of metabolic process | 32.2581 | 4 | no |
| GO:0010604 | positive regulation of macromolecule metabolic process | 32.2581 | 5 | no |
| GO:0010646 | regulation of cell communication | 32.2581 | 4 | no |
| GO:0044087 | regulation of cellular component biogenesis | 29.0323 | 4 | no |
| GO:0048584 | positive regulation of response to stimulus | 29.0323 | 4 | no |
| GO:0051252 | regulation of RNA metabolic process | 29.0323 | 5 | no |
| GO:0006796 | phosphate-containing compound metabolic process | 29.0323 | 4 | no |
| GO:0010629 | negative regulation of gene expression | 29.0323 | 7 | no |
| GO:0009057 | macromolecule catabolic process | 29.0323 | 4 | no |
| GO:0090304 | nucleic acid metabolic process | 29.0323 | 4 | no |
| GO:0009966 | regulation of signal transduction | 29.0323 | 4 | no |
| GO:0010647 | positive regulation of cell communication | 29.0323 | 5 | no |
| GO:0019219 | regulation of nucleobase-containing compound metabolic process | 29.0323 | 5 | no |
| GO:0023056 | positive regulation of signaling | 29.0323 | 4 | no |
| GO:0031399 | regulation of protein modification process | 25.8065 | 6 | no |
| GO:0051246 | regulation of protein metabolic process | 25.8065 | 5 | no |
| GO:0051247 | positive regulation of protein metabolic process | 25.8065 | 6 | no |
| GO:0071702 | organic substance transport | 25.8065 | 4 | no |
| GO:0071705 | nitrogen compound transport | 25.8065 | 4 | no |
| GO:1901565 | organonitrogen compound catabolic process | 25.8065 | 4 | no |
| GO:0043933 | protein-containing complex organization | 25.8065 | 4 | no |
| GO:0009894 | regulation of catabolic process | 25.8065 | 4 | no |
| GO:0009967 | positive regulation of signal transduction | 25.8065 | 5 | no |
| GO:0045862 | positive regulation of proteolysis | 22.5806 | 7 | no |
| GO:1901873 | regulation of post-translational protein modification | 22.5806 | 7 | no |
| GO:1903050 | regulation of proteolysis involved in protein catabolic process | 22.5806 | 7 | no |
| GO:1903052 | positive regulation of proteolysis involved in protein catabolic process | 22.5806 | 8 | no |
| GO:1903320 | regulation of protein modification by small protein conjugation or removal | 22.5806 | 8 | no |
| GO:2000026 | regulation of multicellular organismal development | 22.5806 | 4 | no |
| GO:2001141 | regulation of RNA biosynthetic process | 22.5806 | 6 | no |
| GO:0008104 | protein localization | 22.5806 | 4 | no |
| GO:0051049 | regulation of transport | 22.5806 | 4 | no |
| GO:1902531 | regulation of intracellular signal transduction | 22.5806 | 5 | no |
| GO:0006163 | purine nucleotide metabolic process | 22.5806 | 5 | no |
| GO:0006753 | nucleoside phosphate metabolic process | 22.5806 | 4 | no |
| GO:0009117 | nucleotide metabolic process | 22.5806 | 5 | no |
| GO:0072521 | purine-containing compound metabolic process | 22.5806 | 4 | no |
| GO:0016070 | RNA metabolic process | 22.5806 | 5 | no |
| GO:0043412 | macromolecule modification | 22.5806 | 4 | no |
| GO:0065003 | protein-containing complex assembly | 22.5806 | 5 | no |
| GO:0006355 | regulation of DNA-templated transcription | 22.5806 | 7 | no |
| GO:0009891 | positive regulation of biosynthetic process | 22.5806 | 5 | no |
| GO:0009896 | positive regulation of catabolic process | 22.5806 | 5 | no |
| GO:0010557 | positive regulation of macromolecule biosynthetic process | 22.5806 | 6 | no |
| GO:0010648 | negative regulation of cell communication | 22.5806 | 5 | no |
| GO:0023057 | negative regulation of signaling | 22.5806 | 4 | no |
| GO:0030162 | regulation of proteolysis | 22.5806 | 6 | no |
| GO:0031325 | positive regulation of cellular metabolic process | 22.5806 | 5 | no |
| GO:0031328 | positive regulation of cellular biosynthetic process | 22.5806 | 6 | no |
| GO:0031396 | regulation of protein ubiquitination | 22.5806 | 9 | no |
| GO:0019752 | carboxylic acid metabolic process | 19.3548 | 5 | yes |
| GO:0043436 | oxoacid metabolic process | 19.3548 | 4 | yes |
| GO:0032787 | monocarboxylic acid metabolic process | 16.1290 | 6 | yes |
| GO:0009081 | branched-chain amino acid metabolic process | 12.9032 | 4 | yes |
| GO:0016054 | organic acid catabolic process | 12.9032 | 4 | yes |
| GO:0046395 | carboxylic acid catabolic process | 12.9032 | 5 | yes |
| GO:0006631 | fatty acid metabolic process | 9.6774 | 4 | yes |
| GO:0009062 | fatty acid catabolic process | 6.4516 | 5 | yes |
| GO:0016042 | lipid catabolic process | 6.4516 | 4 | yes |
| GO:0044242 | cellular lipid catabolic process | 6.4516 | 4 | yes |
| GO:0072329 | monocarboxylic acid catabolic process | 6.4516 | 6 | yes |
Disease
No data found.
All partners
| Uniprot ID | Details | Highest evidence | Localizing into PSD | HPA (protein expression in neurons) |
|---|---|---|---|---|
| PCCB_HUMAN | [view interactions] | Low throughput | no | yes |
| UBE2N_HUMAN | [view entry] [view interactions] | Low throughput | yes | yes |
| IFIT1_HUMAN | [view interactions] | High throughput | no | no |
| SIR4_HUMAN | [view interactions] | High throughput | no | yes |
| CDC73_HUMAN | [view interactions] | High throughput | no | yes |
| 2ABB_HUMAN | [view interactions] | High throughput | no | yes |
| GATA4_HUMAN | [view interactions] | High throughput | no | no |
| OSMR_HUMAN | [view interactions] | High throughput | no | yes |
| TLK2_HUMAN | [view interactions] | High throughput | no | no |
| CRCM_HUMAN | [view interactions] | High throughput | no | no |
| BAG2_HUMAN | [view entry] [view interactions] | High throughput | yes | no |
| NFKB1_HUMAN | [view interactions] | High throughput | no | yes |
| PAF1_HUMAN | [view interactions] | High throughput | no | yes |
| PARP2_HUMAN | [view interactions] | High throughput | no | yes |
| FMR1_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| DYRK2_HUMAN | [view interactions] | High throughput | no | yes |
| ISX_HUMAN | [view interactions] | High throughput | no | no |
| VSX1_HUMAN | [view interactions] | High throughput | no | no |
| FHI1A_HUMAN | [view interactions] | High throughput | no | yes |
| FHI2A_HUMAN | [view interactions] | High throughput | no | yes |
| KCNK3_HUMAN | [view interactions] | High throughput | no | no |
| CFTR_HUMAN | [view interactions] | High throughput | no | no |
| SMC5_HUMAN | [view interactions] | High throughput | no | yes |
| ERRFI_HUMAN | [view interactions] | High throughput | no | no |
| PRKN_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| HUWE1_HUMAN | [view interactions] | High throughput | no | yes |
| SYRM_HUMAN | [view interactions] | High throughput | no | yes |
| SGK1_HUMAN | [view interactions] | High throughput | no | yes |
| TSP3_HUMAN | [view interactions] | High throughput | no | yes |
| LEG3_HUMAN | [view interactions] | High throughput | no | yes |
| PTEN_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| IP6K3_HUMAN | [view interactions] | High throughput | no | no |
| CXXC1_HUMAN | [view interactions] | High throughput | no | yes |
| NU155_HUMAN | [view interactions] | High throughput | no | yes |
| HCD2_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| ZN746_HUMAN | [view interactions] | High throughput | no | no |
| ICMT_HUMAN | [view interactions] | High throughput | no | no |
| ACSM5_HUMAN | [view interactions] | High throughput | no | yes |
| TTC1_HUMAN | [view interactions] | High throughput | no | yes |
| NTAQ1_HUMAN | [view interactions] | High throughput | no | yes |
| LAMP3_HUMAN | [view interactions] | High throughput | no | no |
| YBEY_HUMAN | [view interactions] | High throughput | no | yes |
| HNRPL_HUMAN | [view interactions] | High throughput | no | yes |
| CXA1_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| AGR2_HUMAN | [view interactions] | High throughput | no | no |
| RBX1_HUMAN | [view entry] [view interactions] | High throughput | yes | no |
| NR2C2_HUMAN | [view interactions] | High throughput | no | yes |
| H4_HUMAN | [view interactions] | High throughput | no | yes |
| P53_HUMAN | [view interactions] | High throughput | no | no |
| SP100_HUMAN | [view interactions] | High throughput | no | no |
| HNRH1_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| CFAD_HUMAN | [view interactions] | High throughput | no | no |
| FNDC5_HUMAN | [view interactions] | High throughput | no | no |
| PYC_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| MCCA_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| MCCB_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| ACACA_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| RIGI_HUMAN | [view interactions] | High throughput | no | yes |
| TBC25_HUMAN | [view interactions] | High throughput | no | yes |
| FPR1_HUMAN | [view interactions] | High throughput | no | no |
| O52B2_HUMAN | [view interactions] | High throughput | no | no |
| FA81A_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| MASU1_HUMAN | [view interactions] | High throughput | no | yes |
| AQ12B_HUMAN | [view interactions] | High throughput | no | no |
| HDHD3_HUMAN | [view interactions] | High throughput | no | yes |
| TRI43_HUMAN | [view interactions] | High throughput | no | no |
| KBL_HUMAN | [view interactions] | High throughput | no | yes |
| LSMD1_HUMAN | [view interactions] | High throughput | no | no |
| RIPK3_HUMAN | [view interactions] | High throughput | no | no |
| SYYM_HUMAN | [view interactions] | High throughput | no | no |
| MRM2_HUMAN | [view interactions] | High throughput | no | yes |
| KAD4_HUMAN | [view interactions] | High throughput | no | yes |
| CT096_HUMAN | [view interactions] | High throughput | no | yes |
| FAHD1_HUMAN | [view interactions] | High throughput | no | yes |
| UCRI_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| TAZ_HUMAN | [view interactions] | High throughput | no | yes |
| NDUS7_HUMAN | [view entry] [view interactions] | High throughput | yes | no |
| PLPL4_HUMAN | [view interactions] | High throughput | no | yes |
| MTG2_HUMAN | [view interactions] | High throughput | no | yes |
| NPS3A_HUMAN | [view interactions] | High throughput | no | yes |
| RT24_HUMAN | [view interactions] | High throughput | no | yes |
| RM27_HUMAN | [view interactions] | High throughput | no | yes |
| NALP7_HUMAN | [view interactions] | High throughput | no | no |
| PIGR_HUMAN | [view interactions] | High throughput | no | no |
| PLD2_HUMAN | [view interactions] | High throughput | no | yes |
| PLD5_HUMAN | [view interactions] | High throughput | no | no |
| UBP53_HUMAN | [view interactions] | High throughput | no | yes |
| ZRAN1_HUMAN | [view interactions] | High throughput | no | yes |
| HSP7C_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| HS71B_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| HS71A_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| RUFY1_HUMAN | [view interactions] | High throughput | no | yes |
| TRI67_HUMAN | [view interactions] | High throughput | no | no |
| ADA10_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| PML_HUMAN | [view interactions] | High throughput | no | yes |
| PJA1_HUMAN | [view interactions] | High throughput | no | yes |
| MUTA_HUMAN | [view interactions] | High throughput | no | yes |
| DIAP2_HUMAN | [view interactions] | High throughput | no | yes |
| CO6A1_HUMAN | [view interactions] | High throughput | no | no |
| AIDA_HUMAN | [view interactions] | High throughput | no | yes |
| THG1_HUMAN | [view interactions] | High throughput | no | yes |
| GGA1_HUMAN | [view interactions] | High throughput | no | yes |
| PSME2_HUMAN | [view interactions] | High throughput | no | no |
| TRI14_HUMAN | [view interactions] | High throughput | no | yes |
| LTN1_HUMAN | [view interactions] | High throughput | no | no |
| DCA15_HUMAN | [view interactions] | High throughput | no | no |
| DYLT1_HUMAN | [view interactions] | High throughput | no | no |
| DCTN4_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| BICD1_HUMAN | [view interactions] | High throughput | no | yes |
| HOOK1_HUMAN | [view interactions] | High throughput | no | no |
| HOOK3_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| UBC_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| NECD_HUMAN | [view interactions] | High throughput | no | no |
| MK14_HUMAN | [view interactions] | High throughput | no | yes |
| LEG9_HUMAN | [view interactions] | High throughput | no | yes |
| NAA40_HUMAN | [view interactions] | High throughput | no | no |
| CCNF_HUMAN | [view interactions] | High throughput | no | no |
| PER2_HUMAN | [view interactions] | High throughput | no | yes |
| ARF4_HUMAN | [view entry] [view interactions] | High throughput | yes | no |
| ARF5_HUMAN | [view entry] [view interactions] | High throughput | yes | no |
| SAR1A_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| SAR1B_HUMAN | [view interactions] | High throughput | no | yes |
| ARL1_HUMAN | [view interactions] | High throughput | no | yes |
| ARL3_HUMAN | [view interactions] | High throughput | no | yes |
| ARL4D_HUMAN | [view interactions] | High throughput | no | no |
| ARL5A_HUMAN | [view interactions] | High throughput | no | yes |
| ARL5B_HUMAN | [view interactions] | High throughput | no | yes |
| ARL10_HUMAN | [view interactions] | High throughput | no | yes |
| TRI23_HUMAN | [view interactions] | High throughput | no | yes |
| RHEB_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| KDM1A_HUMAN | [view interactions] | High throughput | no | yes |
| HDAC1_HUMAN | [view interactions] | High throughput | no | no |
| PF21A_HUMAN | [view interactions] | High throughput | no | yes |
| RCOR1_HUMAN | [view interactions] | High throughput | no | yes |
| PCDH7_HUMAN | [view entry] [view interactions] | High throughput | yes | no |
| KBTB4_HUMAN | [view interactions] | High throughput | no | yes |
| SND1_HUMAN | [view entry] [view interactions] | High throughput | yes | yes |
| MYC_HUMAN | [view interactions] | High throughput | no | yes |
| DCAF4_HUMAN | [view interactions] | High throughput | no | yes |
| DET1_HUMAN | [view interactions] | High throughput | no | no |
| FBXW5_HUMAN | [view interactions] | High throughput | no | no |
| WDTC1_HUMAN | [view interactions] | High throughput | no | yes |
| RFA1_HUMAN | [view interactions] | High throughput | no | yes |
| RFA2_HUMAN | [view interactions] | High throughput | no | yes |
| RFA3_HUMAN | [view interactions] | High throughput | no | yes |
| YAP1_HUMAN | [view interactions] | High throughput | no | yes |
| SYNPO_HUMAN | [view entry] [view interactions] | High throughput | yes | no |