Protein family
The protein has not catalytic activity
Protein sequence
Protein function
Catalytic activity
| PDB | Resolution (Å) | PDB name |
|---|---|---|
| 6VLZ | 2.97 | Structure of the human mitochondrial ribosome-EF-G1 complex (ClassI) |
| 6VMI | 2.96 | Structure of the human mitochondrial ribosome-EF-G1 complex (ClassIII) |
| 6ZM5 | 2.89 | Human mitochondrial ribosome in complex with OXA1L, mRNA, A/A tRNA, P/P tRNA and nascent polypeptide |
| 6ZM6 | 2.59 | Human mitochondrial ribosome in complex with mRNA, A/A tRNA and P/P tRNA |
| 6ZS9 | 4.0 | Human mitochondrial ribosome in complex with ribosome recycling factor |
| 6ZSA | 4.0 | Human mitochondrial ribosome bound to mRNA, A-site tRNA and P-site tRNA |
| 6ZSB | 4.5 | Human mitochondrial ribosome in complex with mRNA and P-site tRNA |
| 6ZSC | 3.5 | Human mitochondrial ribosome in complex with E-site tRNA |
| 6ZSD | 3.7 | Human mitochondrial ribosome in complex with mRNA, P-site tRNA and E-site tRNA |
| 6ZSE | 5.0 | Human mitochondrial ribosome in complex with mRNA, A/P-tRNA and P/E-tRNA |
| 6ZSG | 4.0 | Human mitochondrial ribosome in complex with mRNA, A-site tRNA, P-site tRNA and E-site tRNA |
| 7L08 | 3.49 | Cryo-EM structure of the human 55S mitoribosome-RRFmt complex. |
| 7L20 | 3.15 | Cryo-EM structure of the human 39S mitoribosomal subunit in complex with RRFmt and EF-G2mt. |
| 7O9K | 3.1 | Human mitochondrial ribosome large subunit assembly intermediate with MTERF4-NSUN4, MRM2, MTG1, the MALSU module, GTPBP5 and mtEF-Tu |
| 7O9M | 2.6 | Human mitochondrial ribosome large subunit assembly intermediate with MTERF4-NSUN4, MRM2, MTG1 and the MALSU module |
| 7OG4 | 3.8 | Human mitochondrial ribosome in complex with P/E-tRNA |
| 7PO2 | 3.09 | Initiation complex of human mitochondrial ribosome small subunit with IF2, fMet-tRNAMet and mRNA |
| 7PO4 | 2.56 | Assembly intermediate of human mitochondrial ribosome large subunit (largely unfolded rRNA with MALSU1, L0R8F8 and ACP) |
| 7QI4 | 2.21 | Human mitochondrial ribosome at 2.2 A resolution |
| GO ontology
|
GO term | GO description |
|---|---|---|
| Biological Process | GO:0006390 | mitochondrial transcription |
| Biological Process | GO:0032543 | mitochondrial translation |
| Biological Process | GO:0045893 | positive regulation of DNA-templated transcription |
| Biological Process | GO:0006412 | translation |
| Molecular Function | GO:0003723 | RNA binding |
| Molecular Function | GO:0003729 | mRNA binding |
| Molecular Function | GO:0003735 | structural constituent of ribosome |
| Cellular Component | GO:0005743 | mitochondrial inner membrane |
| Cellular Component | GO:0005762 | mitochondrial large ribosomal subunit |
| Cellular Component | GO:0005739 | mitochondrion |
| InterPro
|
InterPro name |
|---|---|
| IPR000206 | Ribosomal protein L7/L12 |
| IPR008932 | Ribosomal protein L7/L12, oligomerisation |
| IPR013823 | Ribosomal protein L7/L12, C-terminal |
| IPR014719 | Ribosomal protein L7/L12, C-terminal/adaptor protein ClpS-like |
| IPR036235 | Ribosomal protein L7/L12, oligomerisation domain superfamily |
| Pfam
|
Pfam name |
|---|---|
| PF00542 | Ribosomal protein L7/L12 C-terminal domain |
| PF16320 | Ribosomal protein L7/L12 dimerisation domain |
| Reactome
|
Reactome Name | Node type
|
Reactome Root | Reactome Root Name |
|---|---|---|---|---|
| R-HSA-5368286 | Mitochondrial translation initiation | Leaf | R-HSA-392499 | Metabolism of proteins |
| R-HSA-5389840 | Mitochondrial translation elongation | Leaf | R-HSA-392499 | Metabolism of proteins |
| R-HSA-5419276 | Mitochondrial translation termination | Leaf | R-HSA-392499 | Metabolism of proteins |
| Location
|
ECO term
|
Pubmed |
|---|---|---|
| Mitochondrion | ECO:0000269 | PubMed:8626705 |
| HPO ID
|
HPO name |
|---|---|
| HP:0000007 | Autosomal recessive inheritance |
| HP:0000218 | High palate |
| HP:0000286 | Epicanthus |
| HP:0000311 | Round face |
| HP:0000369 | Low-set ears |
| HP:0000470 | Short neck |
| HP:0000666 | Horizontal nystagmus |
| HP:0001250 | Seizure |
| HP:0001251 | Ataxia |
| HP:0001263 | Global developmental delay |
| HP:0001324 | Muscle weakness |
| HP:0001337 | Tremor |
| HP:0001344 | Absent speech |
| HP:0001508 | Failure to thrive |
| HP:0001695 | Cardiac arrest |
| HP:0002151 | Increased serum lactate |
| HP:0002500 | Abnormal cerebral white matter morphology |
| HP:0008347 | Decreased activity of mitochondrial complex IV |
| HP:0008936 | Axial hypotonia |
| HP:0011923 | Decreased activity of mitochondrial complex I |
| Disease name | MONDO ID
|
ICD10
|
ICD10 chapter
|
OMIM
|
Orphanet
|
|---|---|---|---|---|---|
| combined oxidative phosphorylation deficiency 45 | MONDO:0033533 | E70 | chapter4, Endocrine, nutritional and metabolic diseases | OMIM:618951 | |
| combined oxidative phosphorylation deficiency 45 | MONDO:0033533 | E88 | chapter4, Endocrine, nutritional and metabolic diseases | OMIM:618951 |