KEGG   Homo sapiens (human): 10449
Entry
10449             CDS       T01001                                 
Symbol
ACAA2, DSAEC, T1
Name
(RefSeq) 3-ketoacyl-CoA thiolase, mitochondrial
  KO
K07508  acetyl-CoA acyltransferase 2 [EC:2.3.1.16]
Organism
hsa  Homo sapiens (human)
Pathway
hsa00062  Fatty acid elongation
hsa00071  Fatty acid degradation
hsa00280  Valine, leucine and isoleucine degradation
hsa01100  Metabolic pathways
hsa01212  Fatty acid metabolism
Module
hsa_M00085  Fatty acid elongation in mitochondria
hsa_M00087  beta-Oxidation
Network
nt06020  beta-Oxidation in mitochondria
nt06024  Valine, leucine and isoleucine degradation
  Element
N00804  beta-Oxidation
N00856  Isoleucine degradation
Brite
KEGG Orthology (KO) [BR:hsa00001]
 09100 Metabolism
  09103 Lipid metabolism
   00062 Fatty acid elongation
    10449 (ACAA2)
   00071 Fatty acid degradation
    10449 (ACAA2)
  09105 Amino acid metabolism
   00280 Valine, leucine and isoleucine degradation
    10449 (ACAA2)
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
     10449 (ACAA2)
SSDB
Motif
Pfam: Thiolase_N Thiolase_C ketoacyl-synt IreB
Other DBs
NCBI-GeneID: 10449
NCBI-ProteinID: NP_006102
OMIM: 604770
HGNC: 83
Ensembl: ENSP00000285093.8
UniProt: P42765 B3KNP8
Structure
LinkDB
Position
18:complement(49782164..49813533)
AA seq 397 aa
MALLRGVFVVAAKRTPFGAYGGLLKDFTATDLSEFAAKAALSAGKVSPETVDSVIMGNVL
QSSSDAIYLARHVGLRVGIPKETPALTINRLCGSGFQSIVNGCQEICVKEAEVVLCGGTE
SMSQAPYCVRNVRFGTKLGSDIKLEDSLWVSLTDQHVQLPMAMTAENLAVKHKISREECD
KYALQSQQRWKAANDAGYFNDEMAPIEVKTKKGKQTMQVDEHARPQTTLEQLQKLPPVFK
KDGTVTAGNASGVADGAGAVIIASEDAVKKHNFTPLARIVGYFVSGCDPSIMGIGPVPAI
SGALKKAGLSLKDMDLVEVNEAFAPQYLAVERSLDLDISKTNVNGGAIALGHPLGGSGSR
ITAHLVHELRRRGGKYAVGSACIGGGQGIAVIIQSTA
NT seq 1194 nt   +upstreamnt  +downstreamnt
atggctctgctccgaggtgtgtttgtagttgctgctaagcgaacgccctttggagcttac
ggaggccttctgaaagacttcactgctactgacttgtctgaatttgctgccaaggctgcc
ttgtctgctggcaaagtctcacctgaaacagttgacagtgtgattatgggcaatgtcctg
cagagttcttcagatgctatatatttggcaaggcatgttggtttgcgtgtgggaatccca
aaggagaccccagctctcacgattaataggctctgtggttctggttttcagtccattgtg
aatggatgtcaggaaatttgtgttaaagaagctgaagttgttttatgtggaggaaccgaa
agcatgagccaagctccctactgtgtcagaaatgtgcgttttggaaccaagcttggatca
gatatcaagctggaagattctttatgggtatcattaacagatcagcatgtccagctcccc
atggcaatgactgcagagaatcttgctgtaaaacacaaaataagcagagaagaatgtgac
aaatatgccctgcagtcacagcagagatggaaagctgctaatgatgctggctactttaat
gatgaaatggcaccaattgaagtgaagacaaagaaaggaaaacagacaatgcaggtagac
gagcatgctcggccccaaaccaccctggaacagttacagaaacttcctccagtattcaag
aaagatggaactgttactgcagggaatgcatcgggtgtagctgatggtgctggagctgtt
atcatagctagtgaagatgctgttaagaaacataacttcacaccactggcaagaattgtg
ggctactttgtatctggatgtgatccctctatcatgggtattggtcctgtccctgctatc
agtggggcactgaagaaagcaggactgagtcttaaggacatggatttggtagaggtgaat
gaagcttttgctccccagtacttggctgttgagaggagtttggatcttgacataagtaaa
accaatgtgaatggaggagccattgctttgggtcacccactgggaggatctggatcaaga
attactgcacacctggttcacgaattaaggcgtcgaggtggaaaatatgccgttggatca
gcttgcattggaggtggccaaggtattgctgtcatcattcagagcacagcctga

KEGG   Homo sapiens (human): 3032
Entry
3032              CDS       T01001                                 
Symbol
HADHB, ECHB, MSTP029, MTPB, MTPD, MTPD2, TP-BETA
Name
(RefSeq) trifunctional enzyme subunit beta, mitochondrial isoform 1 precursor
  KO
K07509  acetyl-CoA acyltransferase [EC:2.3.1.16]
Organism
hsa  Homo sapiens (human)
Pathway
hsa00062  Fatty acid elongation
hsa00071  Fatty acid degradation
hsa00280  Valine, leucine and isoleucine degradation
hsa01100  Metabolic pathways
hsa01212  Fatty acid metabolism
Module
hsa_M00085  Fatty acid elongation in mitochondria
hsa_M00087  beta-Oxidation
Network
nt06020  beta-Oxidation in mitochondria
nt06024  Valine, leucine and isoleucine degradation
  Element
N00804  beta-Oxidation
N00856  Isoleucine degradation
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
Pfam: Thiolase_N Thiolase_C ketoacyl-synt Thiolase_C_1
Other DBs
NCBI-GeneID: 3032
NCBI-ProteinID: NP_000174
OMIM: 143450
HGNC: 4803
Ensembl: ENSP00000325136.5
UniProt: P55084
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

KEGG   Homo sapiens (human): 38
Entry
38                CDS       T01001                                 
Symbol
ACAT1, ACAT, MAT, T2, THIL
Name
(RefSeq) acetyl-CoA acetyltransferase, mitochondrial isoform b precursor
  KO
K00626  acetyl-CoA C-acetyltransferase [EC:2.3.1.9]
Organism
hsa  Homo sapiens (human)
Pathway
hsa00071  Fatty acid degradation
hsa00280  Valine, leucine and isoleucine degradation
hsa00310  Lysine degradation
hsa00380  Tryptophan metabolism
hsa00620  Pyruvate metabolism
hsa00630  Glyoxylate and dicarboxylate metabolism
hsa00650  Butanoate metabolism
hsa00900  Terpenoid backbone biosynthesis
hsa01100  Metabolic pathways
hsa01200  Carbon metabolism
hsa01212  Fatty acid metabolism
hsa04975  Fat digestion and absorption
Module
hsa_M00095  C5 isoprenoid biosynthesis, mevalonate pathway
Network
nt06020  beta-Oxidation in mitochondria
nt06034  Cholesterol biosynthesis
  Element
N00804  beta-Oxidation
N01635  Mevalonate pathway
Disease
H01076  Beta-ketothiolase deficiency
Brite
KEGG Orthology (KO) [BR:hsa00001]
 09100 Metabolism
  09101 Carbohydrate metabolism
   00620 Pyruvate metabolism
    38 (ACAT1)
   00630 Glyoxylate and dicarboxylate metabolism
    38 (ACAT1)
   00650 Butanoate metabolism
    38 (ACAT1)
  09103 Lipid metabolism
   00071 Fatty acid degradation
    38 (ACAT1)
  09105 Amino acid metabolism
   00280 Valine, leucine and isoleucine degradation
    38 (ACAT1)
   00310 Lysine degradation
    38 (ACAT1)
   00380 Tryptophan metabolism
    38 (ACAT1)
  09109 Metabolism of terpenoids and polyketides
   00900 Terpenoid backbone biosynthesis
    38 (ACAT1)
 09150 Organismal Systems
  09154 Digestive system
   04975 Fat digestion and absorption
    38 (ACAT1)
 09180 Brite Hierarchies
  09183 Protein families: signaling and cellular processes
   04147 Exosome [BR:hsa04147]
    38 (ACAT1)
Enzymes [BR:hsa01000]
 2. Transferases
  2.3  Acyltransferases
   2.3.1  Transferring groups other than aminoacyl groups
    2.3.1.9  acetyl-CoA C-acetyltransferase
     38 (ACAT1)
Exosome [BR:hsa04147]
 Exosomal proteins
  Exosomal proteins of other body fluids (saliva and urine)
   38 (ACAT1)
SSDB
Motif
Pfam: Thiolase_N Thiolase_C ketoacyl-synt YehY_N
Other DBs
NCBI-GeneID: 38
NCBI-ProteinID: NP_000010
OMIM: 607809
HGNC: 93
Ensembl: ENSP00000265838.4
UniProt: P24752 A0A140VJX1
Structure
LinkDB
Position
11:108116705..108147603
AA seq 427 aa
MAVLAALLRSGARSRSPLLRRLVQEIRYVERSYVSKPTLKEVVIVSATRTPIGSFLGSLS
LLPATKLGSIAIQGAIEKAGIPKEEVKEAYMGNVLQGGEGQAPTRQAVLGAGLPISTPCT
TINKVCASGMKAIMMASQSLMCGHQDVMVAGGMESMSNVPYVMNRGSTPYGGVKLEDLIV
KDGLTDVYNKIHMGSCAENTAKKLNIARNEQDAYAINSYTRSKAAWEAGKFGNEVIPVTV
TVKGQPDVVVKEDEEYKRVDFSKVPKLKTVFQKENGTVTAANASTLNDGAAALVLMTADA
AKRLNVTPLARIVAFADAAVEPIDFPIAPVYAASMVLKDVGLKKEDIAMWEVNEAFSLVV
LANIKMLEIDPQKVNINGGAVSLGHPIGMSGARIVGHLTHALKQGEYGLASICNGGGGAS
AMLIQKL
NT seq 1284 nt   +upstreamnt  +downstreamnt
atggctgtgctggcggcacttctgcgcagcggcgcccgcagccgcagccccctgctccgg
aggctggtgcaggaaataagatatgtggaacggagttatgtatcaaaacccactttgaag
gaagtggtcatagtaagtgctacaagaacacccattggatcttttttaggcagcctttcc
ttgctgccagccactaagcttggttccattgcaattcagggagccattgaaaaggcaggg
attccaaaagaagaagtgaaagaagcatacatgggtaatgttctacaaggaggtgaagga
caagctcctacaaggcaggcagtattgggtgcaggcttacctatttctactccatgtacc
accataaacaaagtttgtgcttcaggaatgaaagccatcatgatggcctctcaaagtctt
atgtgtggacatcaggatgtgatggtggcaggtgggatggagagcatgtccaatgttcca
tatgtaatgaacagaggatcaacaccatatggtggggtaaagcttgaagatttgattgta
aaagacgggctaactgatgtctacaataaaattcatatgggcagctgtgctgagaataca
gcaaagaagctgaatattgcacgaaatgaacaggacgcttatgctattaattcttatacc
agaagtaaagcagcatgggaagctgggaaatttggaaatgaagttattcctgtcacagtt
acagtaaaaggtcaaccagatgtagtggtgaaagaagatgaagaatataaacgtgttgat
tttagcaaagttccaaagctgaagacagttttccagaaagaaaatggcacagtaacagct
gccaatgccagtacactgaatgatggagcagctgctctggttctcatgacggcagatgca
gcgaagaggctcaatgttacaccactggcaagaatagtagcatttgctgacgctgctgta
gaacctattgattttccaattgctcctgtatatgctgcatctatggttcttaaagatgtg
ggattgaaaaaagaagatattgcaatgtgggaagtaaatgaagcctttagtctggttgta
ctagcaaacattaaaatgttggagattgatccccaaaaagtgaatatcaatggaggagct
gtttctctgggacatccaattgggatgtctggagccaggattgttggtcatttgactcat
gccttgaagcaaggagaatacggtcttgccagtatttgcaatggaggaggaggtgcttct
gccatgctaattcagaagctgtag

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