| Identification |
|---|
| HMDB Protein ID
| CDBP00374 |
| Secondary Accession Numbers
| Not Available |
| Name
| Gamma-butyrobetaine dioxygenase |
| Description
| Not Available |
| Synonyms
|
- Gamma-BBH
- Gamma-butyrobetaine hydroxylase
- Gamma-butyrobetaine,2-oxoglutarate dioxygenase
|
| Gene Name
| BBOX1 |
| Protein Type
| Enzyme |
| Biological Properties |
|---|
| General Function
| Involved in iron ion binding |
| Specific Function
| Catalyzes the formation of L-carnitine from gamma-butyrobetaine.
|
| GO Classification
|
| Biological Process |
| carnitine biosynthetic process |
| Cellular Component |
| cytosol |
| actin cytoskeleton |
| mitochondrion |
| intracellular membrane-bounded organelle |
| Function |
| ion binding |
| cation binding |
| metal ion binding |
| binding |
| catalytic activity |
| transition metal ion binding |
| oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, 2-oxoglutarate as one donor, and incorporation of one atom each of oxygen into both donors |
| iron ion binding |
| oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen |
| oxidoreductase activity |
| Molecular Function |
| iron ion binding |
| gamma-butyrobetaine dioxygenase activity |
| zinc ion binding |
| oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen |
| Process |
| metabolic process |
| cellular metabolic process |
| betaine metabolic process |
| carnitine metabolic process |
| carnitine biosynthetic process |
| oxidation reduction |
| cellular amino acid derivative metabolic process |
| cellular biogenic amine metabolic process |
| cellular amino acid and derivative metabolic process |
|
| Cellular Location
|
- Cytoplasm
|
| Pathways
|
| Name | SMPDB/Pathwhiz | KEGG | | Carnitine Synthesis |    | Not Available | | carnitine biosynthesis | Not Available | Not Available |
|
| Gene Properties |
|---|
| Chromosome Location
| 11 |
| Locus
| 11p14.2 |
| SNPs
| BBOX1 |
| Gene Sequence
|
>1164 bp
ATGGCTTGTACCATCCAAAAGGCAGAAGCACTTGACGGGGCTCATTTGATGCAGATCCTC
TGGTATGATGAGGAAGAGTCTCTCTACCCAGCTGTATGGTTGAGAGACAACTGTCCGTGC
TCTGATTGCTACCTGGATTCTGCAAAAGCACGGAAACTTCTAGTGGAAGCTCTTGATGTG
AACATTGGAATTAAAGGCTTGATATTTGACAGAAAAAAGGTGTACATCACATGGCCCGAT
GAGCATTACAGTGAATTCCAGGCTGATTGGCTGAAGAAAAGATGCTTTTCCAAGCAGGCC
AGAGCAAAGCTCCAAAGAGAATTGTTTTTTCCAGAATGCCAATACTGGGGCTCAGAGCTC
CAGCTACCCACTTTGGATTTTGAAGATGTTTTAAGATATGATGAACATGCATACAAGTGG
CTCTCCACCCTCAAGAAAGTAGGCATAGTAAGACTCACCGGAGCATCTGACAAACCAGGA
GAAGTTTCAAAACTTGGGAAAAGGATGGGTTTCCTCTATCTCACATTTTATGGACATACT
TGGCAAGTGCAAGACAAAATCGATGCAAACAATGTGGCTTACACAACTGGGAAGCTAAGC
TTTCACACTGATTATCCAGCCCTCCATCATCCACCTGGGGTTCAGCTTCTTCACTGCATA
AAGCAAACAGTCACAGGGGGTGATTCAGAAATTGTAGATGGGTTTAATGTGTGCCAAAAA
CTAAAGAAAAATAATCCTCAGGCATTCCAGATTTTGTCCTCTACCTTTGTGGACTTTACA
GACATTGGAGTGGATTACTGTGATTTTTCTGTACAATCAAAACATAAAATTATAGAGTTA
GATGATAAAGGCCAAGTGGTTCGCATCAACTTCAATAACGCAACTAGGGACACAATATTT
GATGTACCTGTTGAAAGAGTTCAGCCTTTTTATGCTGCTCTGAAGGAGTTTGTTGACCTC
ATGAACAGCAAAGAATCCAAGTTTACCTTCAAGATGAATCCAGGTGATGTGATTACTTTT
GATAACTGGCGCTTACTTCATGGCCGACGTAGCTATGAAGCAGGAACTGAGATATCCCGC
CATCTAGAAGGAGCTTATGCTGACTGGGATGTGGTCATGTCAAGGCTTCGTATCTTAAGG
CAGAGGGTGGAGAATGGAAACTGA
|
| Protein Properties |
|---|
| Number of Residues
| 387 |
| Molecular Weight
| 44714.6 |
| Theoretical pI
| 6.744 |
| Pfam Domain Function
|
|
| Signals
|
Not Available
|
|
Transmembrane Regions
|
Not Available
|
| Protein Sequence
|
>Gamma-butyrobetaine dioxygenase
MACTIQKAEALDGAHLMQILWYDEEESLYPAVWLRDNCPCSDCYLDSAKARKLLVEALDV
NIGIKGLIFDRKKVYITWPDEHYSEFQADWLKKRCFSKQARAKLQRELFFPECQYWGSEL
QLPTLDFEDVLRYDEHAYKWLSTLKKVGIVRLTGASDKPGEVSKLGKRMGFLYLTFYGHT
WQVQDKIDANNVAYTTGKLSFHTDYPALHHPPGVQLLHCIKQTVTGGDSEIVDGFNVCQK
LKKNNPQAFQILSSTFVDFTDIGVDYCDFSVQSKHKIIELDDKGQVVRINFNNATRDTIF
DVPVERVQPFYAALKEFVDLMNSKESKFTFKMNPGDVITFDNWRLLHGRRSYEAGTEISR
HLEGAYADWDVVMSRLRILRQRVENGN
|
| External Links |
|---|
| GenBank ID Protein
| Not Available |
| UniProtKB/Swiss-Prot ID
| O75936 |
| UniProtKB/Swiss-Prot Entry Name
| BODG_HUMAN |
| PDB IDs
|
|
| GenBank Gene ID
| AF082868 |
| GeneCard ID
| BBOX1 |
| GenAtlas ID
| BBOX1 |
| HGNC ID
| HGNC:964 |
| References |
|---|
| General References
| Not Available |