| Entry |
|
| Symbol |
HADHB, ECHB, MSTP029, MTPB, MTPD, MTPD2, TP-BETA
|
| Name |
(RefSeq) trifunctional enzyme subunit beta, mitochondrial isoform 1 precursor
|
| KO |
|
| Organism |
|
| Pathway |
| hsa00280 | Valine, leucine and isoleucine degradation |
|
| Module |
|
| Network |
nt06020 beta-Oxidation in mitochondria nt06024 Valine, leucine and isoleucine degradation |
| Element |
|
| Disease |
| H00525 | Disorders of mitochondrial fatty-acid oxidation |
| H01352 | Mitochondrial trifunctional protein deficiency |
|
| Brite |
KEGG Orthology (KO) [BR:hsa00001]
09100 Metabolism
09103 Lipid metabolism
00062 Fatty acid elongation
3032 (HADHB)
00071 Fatty acid degradation
3032 (HADHB)
09105 Amino acid metabolism
00280 Valine, leucine and isoleucine degradation
3032 (HADHB)
Enzymes [BR:hsa01000]
2. Transferases
2.3 Acyltransferases
2.3.1 Transferring groups other than aminoacyl groups
2.3.1.16 acetyl-CoA C-acyltransferase
3032 (HADHB)
|
| SSDB |
|
| Motif |
|
| Other DBs |
|
| Structure |
|
| LinkDB |
|
| Position |
2:26244939..26290465
|
| AA seq |
474 aa
MTILTYPFKNLPTASKWALRFSIRPLSCSSQLRAAPAVQTKTKKTLAKPNIRNVVVVDGV
RTPFLLSGTSYKDLMPHDLARAALTGLLHRTSVPKEVVDYIIFGTVIQEVKTSNVAREAA
LGAGFSDKTPAHTVTMACISANQAMTTGVGLIASGQCDVIVAGGVELMSDVPIRHSRKMR
KLMLDLNKAKSMGQRLSLISKFRFNFLAPELPAVSEFSTSETMGHSADRLAAAFAVSRLE
QDEYALRSHSLAKKAQDEGLLSDVVPFKVPGKDTVTKDNGIRPSSLEQMAKLKPAFIKPY
GTVTAANSSFLTDGASAMLIMAEEKALAMGYKPKAYLRDFMYVSQDPKDQLLLGPTYATP
KVLEKAGLTMNDIDAFEFHEAFSGQILANFKAMDSDWFAENYMGRKTKVGLPPLEKFNNW
GGSLSLGHPFGATGCRLVMAAANRLRKEGGQYGLVAACAAGGQGHAMIVEAYPK |
| NT seq |
1425 nt +upstreamnt +downstreamnt
atgactatcttgacttacccctttaaaaatcttcccactgcatcaaaatgggccctcaga
ttttccataagacctctgagctgttcctcccagctacgagctgccccagctgtccagacc
aaaacgaagaagacgttagccaaacccaatataaggaatgttgtggtggtggatggtgtt
cgcactccatttttgctgtctggcacttcatataaagacctgatgccacatgatttggct
agagcagcgcttacgggtttgttgcatcggaccagtgtccctaaggaagtagttgattat
atcatctttggtacagttattcaggaagtgaaaacaagcaatgtggctagagaggctgcc
cttggagctggcttctctgacaagactcctgctcacactgtcaccatggcttgtatctct
gccaaccaagccatgaccacaggtgttggcttgattgcttctggccagtgtgatgtgatc
gtggcaggtggtgttgagttgatgtccgatgtccctattcgtcactcaaggaaaatgaga
aaactgatgcttgatctcaataaggccaaatctatgggccagcgactgtctttaatctct
aaattccgatttaatttcctagcacctgagctccctgcggtttctgagttctccaccagt
gagaccatgggccactctgcagaccgactggccgctgcctttgctgtttctcggctggaa
caggatgaatatgcactgcgctctcacagtctagccaagaaggcacaggatgaaggactc
ctttctgatgtggtacccttcaaagtaccaggaaaagatacagttaccaaagataatggc
atccgtccttcctcactggagcagatggccaaactaaaacctgcattcatcaagccctac
ggcacagtgacagctgcaaattcttctttcttgactgatggtgcatctgcaatgttaatc
atggcggaggaaaaggctctggccatgggttataagccgaaggcatatttgagggatttt
atgtatgtgtctcaggatccaaaagatcaactattacttggaccaacatatgctactcca
aaagttctagaaaaggcaggattgaccatgaatgatattgatgcttttgaatttcatgaa
gctttctcgggtcagattttggcaaattttaaagccatggattctgattggtttgcagaa
aactacatgggtagaaaaaccaaggttggattgcctcctttggagaagtttaataactgg
ggtggatctctgtccctgggacacccatttggagccactggctgcaggttggtcatggct
gctgccaacagattacggaaagaaggaggccagtatggcttagtggctgcgtgtgcagct
ggagggcagggccatgctatgatagtggaagcttatccaaaataa |