Gene/Proteome Database (LMPD)
LMPD ID
LMP006799
Gene ID
Species
Homo sapiens (Human)
Gene Name
lipoyltransferase 1
Gene Symbol
Synonyms
-
Alternate Names
lipoyltransferase 1, mitochondrial; lipoyl ligase; lipoate biosynthesis protein
Chromosome
2
Map Location
2q11.2
EC Number
2.3.1.-
Summary
The process of transferring lipoic acid to proteins is a two-step process. The first step is the activation of lipoic acid by lipoate-activating enzyme to form lipoyl-AMP. For the second step, the protein encoded by this gene transfers the lipoyl moiety to apoproteins. Alternative splicing results in multiple transcript variants. A related pseudogene has been identified on chromosome 13. Read-through transcription also exists between this gene and the neighboring downstream mitochondrial ribosomal protein L30 (MRPL30) gene. [provided by RefSeq, Mar 2011]
Orthologs
Proteins
| lipoyltransferase 1, mitochondrial precursor | |
|---|---|
| Refseq ID | NP_660198 |
| Protein GI | 21729880 |
| UniProt ID | Q9Y234 |
| mRNA ID | NM_145197 |
| Length | 373 |
| RefSeq Status | REVIEWED |
| MLIPFSMKNCFQLLCNCQVPAAGFKKTVKNGLILQSISNDVYQNLAVEDWIHDHMNLEGKPILFFWQNSPSVVIGRHQNPWQECNLNLMREEGIKLARRRSGGGTVYHDMGNINLTFFTTKKKYDRMENLKLIVRALNAVQPQLDVQATKRFDLLLDGQFKISGTASKIGRTTAYHHCTLLCSTDGTFLSSLLKSPYQGIRSNATASIPSLVKNLLEKDPTLTCEVLMNAVATEYAAYHQIDNHIHLINPTDETLFPGINSKAKELQTWEWIYGKTPKFSINTSFHVLYEQSHLEIKVFIDIKNGRIEICNIEAPDHWLPLEIRDKLNSSLIGSKFCPTETTMLTNILLRTCPQDHKLNSKWNILCEKIKGIM | |
| lipoyltransferase 1, mitochondrial precursor | |
|---|---|
| Refseq ID | NP_057013 |
| Protein GI | 7706252 |
| UniProt ID | Q9Y234 |
| mRNA ID | NM_015929 |
| Length | 373 |
| RefSeq Status | REVIEWED |
| Protein sequence is identical to GI:21729880 (mRNA isoform) | |
| lipoyltransferase 1, mitochondrial precursor | |
|---|---|
| Refseq ID | NP_660199 |
| Protein GI | 21729882 |
| UniProt ID | Q9Y234 |
| mRNA ID | NM_145198 |
| Length | 373 |
| RefSeq Status | REVIEWED |
| Protein sequence is identical to GI:21729880 (mRNA isoform) | |
| lipoyltransferase 1, mitochondrial precursor | |
|---|---|
| Refseq ID | NP_001191759 |
| Protein GI | 325651943 |
| UniProt ID | Q9Y234 |
| mRNA ID | NM_001204830 |
| Length | 373 |
| RefSeq Status | REVIEWED |
| Protein sequence is identical to GI:21729880 (mRNA isoform) | |
| lipoyltransferase 1, mitochondrial precursor | |
|---|---|
| Refseq ID | NP_660200 |
| Protein GI | 21729884 |
| UniProt ID | Q9Y234 |
| mRNA ID | NM_145199 |
| Length | 373 |
| RefSeq Status | REVIEWED |
| Protein sequence is identical to GI:21729880 (mRNA isoform) | |
| transit_peptide: 1..25 inference: non-experimental evidence, no additional details recorded note: Mitochondrion (By similarity); propagated from UniProtKB/Swiss-Prot (Q9Y234.1) calculated_mol_wt: 2804 peptide sequence: MLIPFSMKNCFQLLCNCQVPAAGFK mat_peptide: 26..373 product: Lipoyltransferase 1, mitochondrial experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (Q9Y234.1) calculated_mol_wt: 39693 peptide sequence: KTVKNGLILQSISNDVYQNLAVEDWIHDHMNLEGKPILFFWQNSPSVVIGRHQNPWQECNLNLMREEGIKLARRRSGGGTVYHDMGNINLTFFTTKKKYDRMENLKLIVRALNAVQPQLDVQATKRFDLLLDGQFKISGTASKIGRTTAYHHCTLLCSTDGTFLSSLLKSPYQGIRSNATASIPSLVKNLLEKDPTLTCEVLMNAVATEYAAYHQIDNHIHLINPTDETLFPGINSKAKELQTWEWIYGKTPKFSINTSFHVLYEQSHLEIKVFIDIKNGRIEICNIEAPDHWLPLEIRDKLNSSLIGSKFCPTETTMLTNILLRTCPQDHKLNSKWNILCEKIKGIM transit_peptide: 1..25 inference: non-experimental evidence, no additional details recorded note: Mitochondrion (By similarity); propagated from UniProtKB/Swiss-Prot (Q9Y234.1) calculated_mol_wt: 2804 peptide sequence: MLIPFSMKNCFQLLCNCQVPAAGFK mat_peptide: 26..373 product: Lipoyltransferase 1, mitochondrial experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (Q9Y234.1) calculated_mol_wt: 39693 peptide sequence: KTVKNGLILQSISNDVYQNLAVEDWIHDHMNLEGKPILFFWQNSPSVVIGRHQNPWQECNLNLMREEGIKLARRRSGGGTVYHDMGNINLTFFTTKKKYDRMENLKLIVRALNAVQPQLDVQATKRFDLLLDGQFKISGTASKIGRTTAYHHCTLLCSTDGTFLSSLLKSPYQGIRSNATASIPSLVKNLLEKDPTLTCEVLMNAVATEYAAYHQIDNHIHLINPTDETLFPGINSKAKELQTWEWIYGKTPKFSINTSFHVLYEQSHLEIKVFIDIKNGRIEICNIEAPDHWLPLEIRDKLNSSLIGSKFCPTETTMLTNILLRTCPQDHKLNSKWNILCEKIKGIM transit_peptide: 1..25 inference: non-experimental evidence, no additional details recorded note: Mitochondrion (By similarity); propagated from UniProtKB/Swiss-Prot (Q9Y234.1) calculated_mol_wt: 2804 peptide sequence: MLIPFSMKNCFQLLCNCQVPAAGFK mat_peptide: 26..373 product: Lipoyltransferase 1, mitochondrial experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (Q9Y234.1) calculated_mol_wt: 39693 peptide sequence: KTVKNGLILQSISNDVYQNLAVEDWIHDHMNLEGKPILFFWQNSPSVVIGRHQNPWQECNLNLMREEGIKLARRRSGGGTVYHDMGNINLTFFTTKKKYDRMENLKLIVRALNAVQPQLDVQATKRFDLLLDGQFKISGTASKIGRTTAYHHCTLLCSTDGTFLSSLLKSPYQGIRSNATASIPSLVKNLLEKDPTLTCEVLMNAVATEYAAYHQIDNHIHLINPTDETLFPGINSKAKELQTWEWIYGKTPKFSINTSFHVLYEQSHLEIKVFIDIKNGRIEICNIEAPDHWLPLEIRDKLNSSLIGSKFCPTETTMLTNILLRTCPQDHKLNSKWNILCEKIKGIM transit_peptide: 1..25 inference: non-experimental evidence, no additional details recorded note: Mitochondrion (By similarity); propagated from UniProtKB/Swiss-Prot (Q9Y234.1) calculated_mol_wt: 2804 peptide sequence: MLIPFSMKNCFQLLCNCQVPAAGFK mat_peptide: 26..373 product: Lipoyltransferase 1, mitochondrial experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (Q9Y234.1) calculated_mol_wt: 39693 peptide sequence: KTVKNGLILQSISNDVYQNLAVEDWIHDHMNLEGKPILFFWQNSPSVVIGRHQNPWQECNLNLMREEGIKLARRRSGGGTVYHDMGNINLTFFTTKKKYDRMENLKLIVRALNAVQPQLDVQATKRFDLLLDGQFKISGTASKIGRTTAYHHCTLLCSTDGTFLSSLLKSPYQGIRSNATASIPSLVKNLLEKDPTLTCEVLMNAVATEYAAYHQIDNHIHLINPTDETLFPGINSKAKELQTWEWIYGKTPKFSINTSFHVLYEQSHLEIKVFIDIKNGRIEICNIEAPDHWLPLEIRDKLNSSLIGSKFCPTETTMLTNILLRTCPQDHKLNSKWNILCEKIKGIM transit_peptide: 1..25 inference: non-experimental evidence, no additional details recorded note: Mitochondrion (By similarity); propagated from UniProtKB/Swiss-Prot (Q9Y234.1) calculated_mol_wt: 2804 peptide sequence: MLIPFSMKNCFQLLCNCQVPAAGFK mat_peptide: 26..373 product: Lipoyltransferase 1, mitochondrial experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (Q9Y234.1) calculated_mol_wt: 39693 peptide sequence: KTVKNGLILQSISNDVYQNLAVEDWIHDHMNLEGKPILFFWQNSPSVVIGRHQNPWQECNLNLMREEGIKLARRRSGGGTVYHDMGNINLTFFTTKKKYDRMENLKLIVRALNAVQPQLDVQATKRFDLLLDGQFKISGTASKIGRTTAYHHCTLLCSTDGTFLSSLLKSPYQGIRSNATASIPSLVKNLLEKDPTLTCEVLMNAVATEYAAYHQIDNHIHLINPTDETLFPGINSKAKELQTWEWIYGKTPKFSINTSFHVLYEQSHLEIKVFIDIKNGRIEICNIEAPDHWLPLEIRDKLNSSLIGSKFCPTETTMLTNILLRTCPQDHKLNSKWNILCEKIKGIM | |
Gene Information
Gene Ontology (GO Annotations)
| GO ID | Source | Type | Description |
|---|---|---|---|
| GO:0005739 | IEA:UniProtKB-KW | C | mitochondrion |
| GO:0016746 | IEA:UniProtKB-KW | F | transferase activity, transferring acyl groups |
| GO:0006464 | TAS:ProtInc | P | cellular protein modification process |
| GO:0006629 | TAS:ProtInc | P | lipid metabolic process |
| GO:0009249 | IEA:InterPro | P | protein lipoylation |
KEGG Pathway Links
| KEGG Pathway ID | Description |
|---|---|
| hsa00785 | Lipoic acid metabolism |
| ko00785 | Lipoic acid metabolism |
| hsa01100 | Metabolic pathways |
BIOCYC Pathway Links
| BIOCYC Pathway ID | Description |
|---|---|
| PWY0-1275 | lipoate biosynthesis and incorporation II |
Domain Information
UniProt Annotations
Entry Information
Comments
| Comment Type | Description |
|---|---|
| Catalytic Activity | Lipoyl-AMP + protein = protein N(6)- (lipoyl)lysine + AMP. |
| Function | Catalyzes the transfer of the lipoyl group from lipoyl- AMP to the specific lysine residue of lipoyl domains of lipoate- dependent enzymes. |
| Pathway | Protein modification; protein lipoylation via exogenous pathway; protein N(6)-(lipoyl)lysine from lipoate: step 2/2. |
| Similarity | Belongs to the LplA family. |
| Similarity | Contains 1 BPL/LPL catalytic domain. |
| Subcellular Location | Mitochondrion. |
| Tissue Specificity | Highly expressed in skeletal muscle and heart, moderately in kidney and pancreas, and detected at lower levels in liver, brain, placenta and lung. |
Identical and Related Proteins
Unique RefSeq proteins for LMP006799 (as displayed in Record Overview)
| Protein GI | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| 21729880 | RefSeq | NP_660198 | 373 | lipoyltransferase 1, mitochondrial precursor |
Identical Sequences to LMP006799 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:21729880 | GenBank | AAX88927.1 | 373 | unknown [Homo sapiens] |
| GI:21729880 | GenBank | EAX01877.1 | 373 | hCG1989457, isoform CRA_b [Homo sapiens] |
| GI:21729880 | GenBank | ADQ32754.1 | 373 | lipoyltransferase 1, partial [synthetic construct] |
| GI:21729880 | GenBank | AIC56486.1 | 373 | LIPT1, partial [synthetic construct] |
| GI:21729880 | GenBank | AIC63432.1 | 373 | LIPT1, partial [synthetic construct] |
| GI:21729880 | RefSeq | NP_001191759.1 | 373 | lipoyltransferase 1, mitochondrial precursor [Homo sapiens] |
Related Sequences to LMP006799 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:21729880 | RefSeq | XP_004031528.1 | 373 | PREDICTED: lipoyltransferase 1, mitochondrial isoform 2 [Gorilla gorilla gorilla] |
| GI:21729880 | RefSeq | XP_004031529.1 | 373 | PREDICTED: lipoyltransferase 1, mitochondrial isoform 3 [Gorilla gorilla gorilla] |
| GI:21729880 | RefSeq | XP_004031531.1 | 373 | PREDICTED: lipoyltransferase 1, mitochondrial isoform 5 [Gorilla gorilla gorilla] |
| GI:21729880 | RefSeq | XP_004031532.1 | 373 | PREDICTED: lipoyltransferase 1, mitochondrial isoform 6 [Gorilla gorilla gorilla] |
| GI:21729880 | RefSeq | XP_004031533.1 | 373 | PREDICTED: lipoyltransferase 1, mitochondrial isoform 7 [Gorilla gorilla gorilla] |
| GI:21729880 | RefSeq | XP_004031534.1 | 373 | PREDICTED: lipoyltransferase 1, mitochondrial isoform 8 [Gorilla gorilla gorilla] |