PCCA_HUMAN
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]
To display all evidence describing the interaction with a partner, click on the protein name left to the bars.
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) | - |
No annotated instance was found. To search for linear motifs, use the ELM prediction server.
Molecular function
Term | Name | % | Distance from the top of the tree | Annotated in PCCA_HUMAN |
---|---|---|---|---|
GO:0017076 | purine nucleotide binding | 33.3333 | 4 | yes |
GO:0030554 | adenyl nucleotide binding | 33.3333 | 5 | yes |
GO:0046872 | metal ion binding | 33.3333 | 4 | yes |
GO:0032555 | purine ribonucleotide binding | 29.6296 | 4 | yes |
GO:0032559 | adenyl ribonucleotide binding | 29.6296 | 5 | yes |
GO:0003723 | RNA binding | 25.9259 | 4 | no |
GO:0005524 | ATP binding | 22.2222 | 5 | yes |
GO:0035639 | purine ribonucleoside triphosphate binding | 22.2222 | 4 | yes |
GO:0031625 | ubiquitin protein ligase binding | 22.2222 | 5 | no |
GO:0044389 | ubiquitin-like protein ligase binding | 22.2222 | 4 | no |
GO:0016421 | CoA carboxylase activity | 14.8148 | 4 | yes |
GO:0031406 | carboxylic acid binding | 14.8148 | 4 | yes |
GO:0033293 | monocarboxylic acid binding | 14.8148 | 5 | yes |
Biological process
Term | Name | % | Distance from top the of the tree | Annotated in PCCA_HUMAN |
---|---|---|---|---|
GO:0019752 | carboxylic acid metabolic process | 44.4444 | 5 | yes |
GO:0043436 | oxoacid metabolic process | 44.4444 | 4 | yes |
GO:0016054 | organic acid catabolic process | 37.0370 | 4 | yes |
GO:0046395 | carboxylic acid catabolic process | 37.0370 | 5 | yes |
GO:0032787 | monocarboxylic acid metabolic process | 33.3333 | 6 | yes |
GO:1901565 | organonitrogen compound catabolic process | 33.3333 | 4 | no |
GO:0060255 | regulation of macromolecule metabolic process | 33.3333 | 4 | no |
GO:0022607 | cellular component assembly | 29.6296 | 4 | no |
GO:0010468 | regulation of gene expression | 29.6296 | 5 | no |
GO:0009063 | cellular amino acid catabolic process | 25.9259 | 4 | no |
GO:0009081 | branched-chain amino acid metabolic process | 25.9259 | 4 | no |
GO:0009083 | branched-chain amino acid catabolic process | 25.9259 | 5 | no |
GO:0043412 | macromolecule modification | 25.9259 | 4 | no |
GO:0048522 | positive regulation of cellular process | 25.9259 | 4 | no |
GO:0009893 | positive regulation of metabolic process | 25.9259 | 4 | no |
GO:0010604 | positive regulation of macromolecule metabolic process | 25.9259 | 5 | no |
GO:0051171 | regulation of nitrogen compound metabolic process | 25.9259 | 4 | no |
GO:0080090 | regulation of primary metabolic process | 25.9259 | 4 | no |
GO:0006631 | fatty acid metabolic process | 22.2222 | 4 | yes |
GO:0006996 | organelle organization | 22.2222 | 4 | no |
GO:0036211 | protein modification process | 22.2222 | 4 | no |
GO:0010646 | regulation of cell communication | 22.2222 | 4 | no |
GO:0031323 | regulation of cellular metabolic process | 22.2222 | 4 | no |
GO:0031399 | regulation of protein modification process | 22.2222 | 6 | no |
GO:0048523 | negative regulation of cellular process | 22.2222 | 4 | no |
GO:0051173 | positive regulation of nitrogen compound metabolic process | 22.2222 | 5 | no |
GO:0051246 | regulation of protein metabolic process | 22.2222 | 5 | no |
GO:0051247 | positive regulation of protein metabolic process | 22.2222 | 6 | no |
GO:0009892 | negative regulation of metabolic process | 22.2222 | 4 | no |
GO:0010605 | negative regulation of macromolecule metabolic process | 22.2222 | 5 | no |
GO:0009062 | fatty acid catabolic process | 18.5185 | 5 | yes |
GO:0016042 | lipid catabolic process | 18.5185 | 4 | yes |
GO:0044242 | cellular lipid catabolic process | 18.5185 | 4 | yes |
GO:0072329 | monocarboxylic acid catabolic process | 18.5185 | 6 | yes |
Disease
No data found.
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 |
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 |
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 |
DDX58_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 |
PJA1_HUMAN | [view interactions] | High throughput | no | yes |
MUTA_HUMAN | [view interactions] | High throughput | no | yes |
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 |
PML_HUMAN | [view interactions] | High throughput | no | yes |
SYNPO_HUMAN | [view entry] [view interactions] | High throughput | yes | no |
ACSA_HUMAN | [view interactions] | Computational | no | no |
ODBB_HUMAN | [view interactions] | Computational | no | yes |
ODBA_HUMAN | [view interactions] | Computational | no | yes |
GABT_HUMAN | [view interactions] | Computational | no | yes |
GRPE1_HUMAN | [view interactions] | Computational | no | yes |
THIK_HUMAN | [view entry] [view interactions] | Computational | yes | yes |
AGT2_HUMAN | [view interactions] | Computational | no | no |
ACS2L_HUMAN | [view interactions] | Computational | no | yes |
ODB2_HUMAN | [view entry] [view interactions] | Computational | yes | yes |
3HIDH_HUMAN | [view interactions] | Computational | no | yes |
MCEE_HUMAN | [view interactions] | Computational | no | no |
ACSS3_HUMAN | [view interactions] | Computational | no | no |
THIM_HUMAN | [view interactions] | Computational | no | yes |
ECHP_HUMAN | [view interactions] | Computational | no | no |
MMSA_HUMAN | [view entry] [view interactions] | Computational | yes | no |
RAB28_HUMAN | [view interactions] | Computational | no | yes |
RBSK_HUMAN | [view interactions] | Computational | no | yes |
BPL1_HUMAN | [view interactions] | Computational | no | yes |
DLDH_HUMAN | [view entry] [view interactions] | Computational | yes | yes |
ECHD1_HUMAN | [view interactions] | Computational | no | no |
AOXA_HUMAN | [view interactions] | Computational | no | no |
THIL_HUMAN | [view entry] [view interactions] | Computational | yes | yes |
ACOX1_HUMAN | [view interactions] | Computational | no | yes |
ACADS_HUMAN | [view interactions] | Computational | no | yes |
ECHB_HUMAN | [view entry] [view interactions] | Computational | yes | yes |
ACOX3_HUMAN | [view interactions] | Computational | no | yes |