| Identification |
| HMDB Protein ID
| CDBP00046 |
| Secondary Accession Numbers
| Not Available |
| Name
| Trifunctional enzyme subunit beta, mitochondrial |
| Description
| Not Available |
| Synonyms
|
- 3-ketoacyl-CoA thiolase
- Acetyl-CoA acyltransferase
- Beta-ketothiolase
- TP-beta
|
| Gene Name
| HADHB |
| Protein Type
| Enzyme |
| Biological Properties |
| General Function
| Involved in transferase activity, transferring acyl groups other than amino-acyl groups |
| Specific Function
| Not Available |
| GO Classification
|
| Biological Process |
| cardiolipin acyl-chain remodeling |
| glycerophospholipid biosynthetic process |
| fatty acid beta-oxidation |
| Cellular Component |
| endoplasmic reticulum |
| mitochondrial outer membrane |
| mitochondrial nucleoid |
| mitochondrial fatty acid beta-oxidation multienzyme complex |
| mitochondrial inner membrane |
| Function |
| catalytic activity |
| transferase activity |
| transferase activity, transferring acyl groups |
| transferase activity, transferring acyl groups other than amino-acyl groups |
| Molecular Function |
| enoyl-CoA hydratase activity |
| long-chain-3-hydroxyacyl-CoA dehydrogenase activity |
| acetyl-CoA C-acyltransferase activity |
| NAD binding |
| long-chain-enoyl-CoA hydratase activity |
| fatty-acyl-CoA binding |
| 3-hydroxyacyl-CoA dehydrogenase activity |
| Process |
| metabolic process |
|
| Cellular Location
|
- Mitochondrion
|
| Pathways
|
| Name | SMPDB/Pathwhiz | KEGG | | fatty acid beta-oxidation | Not Available | Not Available | | Fatty acid elongation | Not Available |  | | Fatty Acid Elongation In Mitochondria |    |  | | Long-chain-3-hydroxyacyl-coa dehydrogenase deficiency (LCHAD) |    | Not Available | | Fatty acid Metabolism |    |  |
|
| Gene Properties |
| Chromosome Location
| 2 |
| Locus
| 2p23 |
| SNPs
| HADHB |
| Gene Sequence
|
>1425 bp
ATGACTATCTTGACTTACCCCTTTAAAAATCTTCCCACTGCATCAAAATGGGCCCTCAGA
TTTTCCATAAGACCTCTGAGCTGTTCCTCCCAGCTACGAGCTGCCCCAGCTGTCCAGACC
AAAACGAAGAAGACGTTAGCCAAACCCAATATAAGGAATGTTGTGGTGGTGGATGGTGTT
CGCACTCCATTTTTGCTGTCTGGCACTTCATATAAAGACCTGATGCCACATGATTTGGCT
AGAGCAGCGCTTACGGGTTTGTTGCATCGGACCAGTGTCCCTAAGGAAGTAGTTGATTAT
ATCATCTTTGGTACAGTTATTCAGGAAGTGAAAACAAGCAATGTGGCTAGAGAGGCTGCC
CTTGGAGCTGGCTTCTCTGACAAGACTCCTGCTCACACTGTCACCATGGCTTGTATCTCT
GCCAACCAAGCCATGACCACAGGTGTTGGCTTGATTGCTTCTGGCCAGTGTGATGTGATC
GTGGCAGGTGGTGTTGAGTTGATGTCCGATGTCCCTATTCGTCACTCAAGGAAAATGAGA
AAACTGATGCTTGATCTCAATAAGGCCAAATCTATGGGCCAGCGACTGTCTTTAATCTCT
AAATTCCGATTTAATTTCCTAGCACCTGAGCTCCCTGCGGTTTCTGAGTTCTCCACCAGT
GAGACCATGGGCCACTCTGCAGACCGACTGGCCGCTGCCTTTGCTGTTTCTCGGCTGGAA
CAGGATGAATATGCACTGCGCTCTCACAGTCTAGCCAAGAAGGCACAGGATGAAGGACTC
CTTTCTGATGTGGTACCCTTCAAAGTACCAGGAAAAGATACAGTTACCAAAGATAATGGC
ATCCGTCCTTCCTCACTGGAGCAGATGGCCAAACTAAAACCTGCATTCATCAAGCCCTAC
GGCACAGTGACAGCTGCAAATTCTTCTTTCTTGACTGATGGTGCATCTGCAATGTTAATC
ATGGCGGAGGAAAAGGCTCTGGCCATGGGTTATAAGCCGAAGGCATATTTGAGGGATTTT
ATGTATGTGTCTCAGGATCCAAAAGATCAACTATTACTTGGACCAACATATGCTACTCCA
AAAGTTCTAGAAAAGGCAGGATTGACCATGAATGATATTGATGCTTTTGAATTTCATGAA
GCTTTCTCGGGTCAGATTTTGGCAAATTTTAAAGCCATGGATTCTGATTGGTTTGCAGAA
AACTACATGGGTAGAAAAACCAAGGTTGGATTGCCTCCTTTGGAGAAGTTTAATAACTGG
GGTGGATCTCTGTCCCTGGGACACCCATTTGGAGCCACTGGCTGCAGGTTGGTCATGGCT
GCTGCCAACAGATTACGGAAAGAAGGAGGCCAGTATGGCTTAGTGGCTGCGTGTGCAGCT
GGAGGGCAGGGCCATGCTATGATAGTGGAAGCTTATCCAAAATAA
|
| Protein Properties |
| Number of Residues
| 474 |
| Molecular Weight
| 51293.955 |
| Theoretical pI
| 9.406 |
| Pfam Domain Function
|
|
| Signals
|
Not Available
|
|
Transmembrane Regions
|
Not Available
|
| Protein Sequence
|
>Trifunctional enzyme subunit beta, mitochondrial
MTILTYPFKNLPTASKWALRFSIRPLSCSSQLRAAPAVQTKTKKTLAKPNIRNVVVVDGV
RTPFLLSGTSYKDLMPHDLARAALTGLLHRTSVPKEVVDYIIFGTVIQEVKTSNVAREAA
LGAGFSDKTPAHTVTMACISANQAMTTGVGLIASGQCDVIVAGGVELMSDVPIRHSRKMR
KLMLDLNKAKSMGQRLSLISKFRFNFLAPELPAVSEFSTSETMGHSADRLAAAFAVSRLE
QDEYALRSHSLAKKAQDEGLLSDVVPFKVPGKDTVTKDNGIRPSSLEQMAKLKPAFIKPY
GTVTAANSSFLTDGASAMLIMAEEKALAMGYKPKAYLRDFMYVSQDPKDQLLLGPTYATP
KVLEKAGLTMNDIDAFEFHEAFSGQILANFKAMDSDWFAENYMGRKTKVGLPPLEKFNNW
GGSLSLGHPFGATGCRLVMAAANRLRKEGGQYGLVAACAAGGQGHAMIVEAYPK
|
| External Links |
| GenBank ID Protein
| Not Available |
| UniProtKB/Swiss-Prot ID
| P55084 |
| UniProtKB/Swiss-Prot Entry Name
| ECHB_HUMAN |
| PDB IDs
|
Not Available |
| GenBank Gene ID
| D16481 |
| GeneCard ID
| HADHB |
| GenAtlas ID
| HADHB |
| HGNC ID
| HGNC:4803 |
| References |
| General References
| Not Available |