KEGG   Homo sapiens (human): 56922
Entry
56922             CDS       T01001                                 
Symbol
MCCC1, MCC-B, MCCA, MCCCalpha
Name
(RefSeq) methylcrotonyl-CoA carboxylase subunit 1
  KO
K01968  3-methylcrotonyl-CoA carboxylase alpha subunit [EC:6.4.1.4]
Organism
hsa  Homo sapiens (human)
Pathway
hsa00280  Valine, leucine and isoleucine degradation
hsa01100  Metabolic pathways
Module
hsa_M00036  Leucine degradation, leucine => acetoacetate + acetyl-CoA
Network
nt06024  Valine, leucine and isoleucine degradation
  Element
N00851  Leucine degradation
Disease
H00181  3-Methylcrotonylglycinuria
Brite
KEGG Orthology (KO) [BR:hsa00001]
 09100 Metabolism
  09105 Amino acid metabolism
   00280 Valine, leucine and isoleucine degradation
    56922 (MCCC1)
Enzymes [BR:hsa01000]
 6. Ligases
  6.4  Forming carbon-carbon bonds
   6.4.1  Ligases that form carbon-carbon bonds (only sub-subclass identified to date)
    6.4.1.4  methylcrotonoyl-CoA carboxylase
     56922 (MCCC1)
SSDB
Motif
Pfam: CPSase_L_D2 Biotin_carb_N Biotin_carb_C Biotin_lipoyl Dala_Dala_lig_C ATP-grasp Biotin_lipoyl_2 RimK ATP-grasp_3 ATPgrasp_ST GARS_A ATP-grasp_4 RnfC_N NQRA 2OG-FeII_Oxy_2 MCCA_BT
Other DBs
NCBI-GeneID: 56922
NCBI-ProteinID: NP_064551
OMIM: 609010
HGNC: 6936
Ensembl: ENSG00000078070
UniProt: Q96RQ3 A0A0S2Z693
Structure
LinkDB
Position
3:complement(183015218..183116196)
AA seq 725 aa
MAAASAVSVLLVAAERNRWHRLPSLLLPPRTWVWRQRTMKYTTATGRNITKVLIANRGEI
ACRVMRTAKKLGVQTVAVYSEADRNSMHVDMADEAYSIGPAPSQQSYLSMEKIIQVAKTS
AAQAIHPGCGFLSENMEFAELCKQEGIIFIGPPPSAIRDMGIKSTSKSIMAAAGVPVVEG
YHGEDQSDQCLKEHARRIGYPVMIKAVRGGGGKGMRIVRSEQEFQEQLESARREAKKSFN
DDAMLIEKFVDTPRHVEVQVFGDHHGNAVYLFERDCSVQRRHQKIIEEAPAPGIKSEVRK
KLGEAAVRAAKAVNYVGAGTVEFIMDSKHNFCFMEMNTRLQVEHPVTEMITGTDLVEWQL
RIAAGEKIPLSQEEITLQGHAFEARIYAEDPSNNFMPVAGPLVHLSTPRADPSTRIETGV
RQGDEVSVHYDPMIAKLVVWAADRQAALTKLRYSLRQYNIVGLHTNIDFLLNLSGHPEFE
AGNVHTDFIPQHHKQLLLSRKAAAKESLCQAALGLILKEKAMTDTFTLQAHDQFSPFSSS
SGRRLNISYTRNMTLKDGKNNVAIAVTYNHDGSYSMQIEDKTFQVLGNLYSEGDCTYLKC
SVNGVASKAKLIILENTIYLFSKEGSIEIDIPVPKYLSSVSSQETQGGPLAPMTGTIEKV
FVKAGDKVKAGDSLMVMIAMKMEHTIKSPKDGTVKKVFYREGAQANRHTPLVEFEEEESD
KRESE
NT seq 2178 nt   +upstreamnt  +downstreamnt
atggcggcggcctctgcggtgtcggtgctgctggtggcggcggagaggaaccggtggcat
cgtctcccgagcctgctcctgccgccgaggacatgggtgtggaggcaaagaaccatgaag
tacacaacagccacaggaagaaacattaccaaggtcctcattgcaaacagaggagaaatt
gcctgcagggtgatgcgcacagccaaaaaactgggtgtacagactgtggcggtttatagt
gaggctgacagaaattccatgcatgtagatatggcagatgaagcatattccatcggcccc
gctccctcccagcagagctacctatctatggagaaaatcattcaagtggccaagacctct
gctgcacaggctatccatccaggatgcggttttctctcagaaaacatggaatttgctgaa
ctttgtaagcaagaaggaattatttttataggccctcctccatctgcaattagagacatg
ggtataaagagcacatccaaatccataatggctgctgctggagtacctgttgtggagggt
tatcatggtgaggaccaatcagaccagtgcctgaaggaacacgccaggagaattggctat
cctgtcatgattaaagccgtccggggtggaggaggaaaaggaatgaggattgttagatca
gaacaagaatttcaagaacagttagagtcagcacggagagaagctaagaagtctttcaat
gatgatgctatgctgatcgagaagtttgtagacacaccgaggcatgtagaagtccaggtg
tttggtgatcaccatggcaatgctgtgtacttgtttgaaagagactgtagtgtgcagagg
cgacatcagaagatcattgaggaggccccagcgcctggtattaaatctgaagtaagaaaa
aagctgggagaagctgcagtcagagctgctaaagctgtaaattatgttggagcagggact
gtggagtttattatggactcaaaacataatttctgtttcatggagatgaatacaaggctg
caagtggaacatcctgttactgagatgatcacaggaactgacttggtggagtggcagctt
agaattgcagcaggagagaagattcctttgagccaggaagaaataactctgcagggccat
gccttcgaagctagaatatatgcagaagatcctagcaataacttcatgcctgtggcaggc
ccattagtgcacctctctactcctcgagcagacccttccaccaggattgaaactggagta
cggcaaggagacgaagtttccgtgcattatgaccccatgattgcgaagctggtcgtgtgg
gcagcagatcgccaggcggcattgacaaaactgaggtacagccttcgtcagtacaatatt
gttggactgcacaccaacattgacttcttactcaacctgtctggccacccagagtttgaa
gctgggaacgtgcacactgatttcatccctcaacaccacaaacagttgttgctcagtcgg
aaggctgcagccaaagagtctttatgccaggcagccctgggtctcatcctcaaggagaaa
gccatgaccgacactttcactcttcaggcacatgatcaattctctccattttcgtctagc
agtggaagaagactgaatatctcgtataccagaaacatgactcttaaagatggtaaaaac
aatgtagccatagctgtaacgtataaccatgatgggtcttatagcatgcagattgaagat
aaaactttccaagtccttggtaatctttacagcgagggagactgcacttacctgaaatgt
tctgttaatggagttgctagtaaagcgaagctgattatcctggaaaacactatttaccta
ttttccaaggaaggaagtattgagattgacattccagtccccaaatacttatcttctgtg
agctcacaagaaactcagggcggccccttagctcctatgactggaaccattgaaaaggtg
tttgtcaaagctggagacaaagtgaaagcgggagattccctcatggttatgatcgccatg
aagatggagcataccataaagtctccaaaggatggcacagtaaagaaagtgttctacaga
gaaggtgctcaggccaacagacacactcctttagtcgagtttgaggaggaagaatcagac
aaaagggaatcggaataa

KEGG   Homo sapiens (human): 64087
Entry
64087             CDS       T01001                                 
Symbol
MCCC2, MCCB, MCCCbeta
Name
(RefSeq) methylcrotonyl-CoA carboxylase subunit 2
  KO
K01969  3-methylcrotonyl-CoA carboxylase beta subunit [EC:6.4.1.4]
Organism
hsa  Homo sapiens (human)
Pathway
hsa00280  Valine, leucine and isoleucine degradation
hsa01100  Metabolic pathways
Module
hsa_M00036  Leucine degradation, leucine => acetoacetate + acetyl-CoA
Network
nt06024  Valine, leucine and isoleucine degradation
  Element
N00851  Leucine degradation
Disease
H00181  3-Methylcrotonylglycinuria
Brite
KEGG Orthology (KO) [BR:hsa00001]
 09100 Metabolism
  09105 Amino acid metabolism
   00280 Valine, leucine and isoleucine degradation
    64087 (MCCC2)
Enzymes [BR:hsa01000]
 6. Ligases
  6.4  Forming carbon-carbon bonds
   6.4.1  Ligases that form carbon-carbon bonds (only sub-subclass identified to date)
    6.4.1.4  methylcrotonoyl-CoA carboxylase
     64087 (MCCC2)
SSDB
Motif
Pfam: Carboxyl_trans ACCA MdcE
Other DBs
NCBI-GeneID: 64087
NCBI-ProteinID: NP_071415
OMIM: 609014
HGNC: 6937
Ensembl: ENSG00000131844
UniProt: Q9HCC0 A0A140VK29
Structure
LinkDB
Position
5:71587340..71658706
AA seq 563 aa
MWAVLRLALRPCARASPAGPRAYHGDSVASLGTQPDLGSALYQENYKQMKALVNQLHERV
EHIKLGGGEKARALHISRGKLLPRERIDNLIDPGSPFLELSQFAGYQLYDNEEVPGGGII
TGIGRVSGVECMIIANDATVKGGAYYPVTVKKQLRAQEIAMQNRLPCIYLVDSGGAYLPR
QADVFPDRDHFGRTFYNQAIMSSKNIAQIAVVMGSCTAGGAYVPAMADENIIVRKQGTIF
LAGPPLVKAATGEEVSAEDLGGADLHCRKSGVSDHWALDDHHALHLTRKVVRNLNYQKKL
DVTIEPSEEPLFPADELYGIVGANLKRSFDVREVIARIVDGSRFTEFKAFYGDTLVTGFA
RIFGYPVGIVGNNGVLFSESAKKGTHFVQLCCQRNIPLLFLQNITGFMVGREYEAEGIAK
DGAKMVAAVACAQVPKITLIIGGSYGAGNYGMCGRAYSPRFLYIWPNARISVMGGEQAAN
VLATITKDQRAREGKQFSSADEAALKEPIIKKFEEEGNPYYSSARVWDDGIIDPADTRLV
LGLSFSAALNAPIEKTDFGIFRM
NT seq 1692 nt   +upstreamnt  +downstreamnt
atgtgggccgtcctgaggttagccctgcggccgtgtgcccgcgcctctcccgccgggccg
cgcgcctatcacggggactcggtggcctcgctgggcacccagccggacttgggctctgcc
ctctaccaggagaactacaagcagatgaaagcactagtaaatcagctccatgaacgagtg
gagcatataaaactaggaggtggtgagaaagcccgagcacttcacatatcaagaggaaaa
ctattgcccagagaaagaattgacaatctcatagacccagggtctccatttctggaatta
tcccagtttgcaggttaccagttatatgacaatgaggaggtgccaggaggtggcattatt
acaggcattggaagagtatcaggagtagaatgcatgattattgccaatgatgccaccgtc
aaaggaggtgcctactacccagtgactgtgaaaaaacaattacgggcccaagaaattgcc
atgcaaaacaggctcccctgcatctacttagttgattcgggaggagcatacttacctcga
caagcagatgtgtttccagatcgagaccactttggccgtacattctataatcaggcaatt
atgtcttctaaaaatattgcacagatcgcagtggtcatgggctcctgcaccgcaggagga
gcctatgtgcctgccatggctgatgaaaacatcattgtacgcaagcagggtaccattttc
ttggcaggaccccccttggttaaagcggcaactggggaagaagtatctgctgaggatctt
ggaggtgctgatcttcattgcagaaagtctggagtaagtgaccactgggctttggatgat
catcatgcccttcacttaactaggaaggttgtgaggaatctaaattatcagaagaaattg
gatgtcaccattgaaccttctgaagagcctttatttcctgctgatgaattgtatggaata
gttggtgctaaccttaagaggagctttgatgtccgagaggtcattgctagaatcgtggat
ggaagcagattcactgagttcaaagccttttatggagacacattagttacaggatttgct
cgaatatttgggtacccagtaggtatcgttggaaacaacggagttctcttttctgaatct
gcaaaaaagggtactcactttgtccagttatgctgccaaagaaatattcctctgctgttc
cttcaaaacattactggatttatggttggtagagagtatgaagctgaaggaattgccaag
gatggtgccaagatggtggccgctgtggcctgtgcccaagtgcctaagataaccctcatc
attgggggctcctatggagccggaaactatgggatgtgtggcagagcatatagcccaaga
tttctctacatttggccaaatgctcgtatctcagtgatgggaggagagcaggcagccaat
gtgttggccacgataacaaaggaccaaagagcccgggaaggaaagcagttctccagtgct
gatgaagcggctttaaaagagcccatcattaagaagtttgaagaggaaggaaacccttac
tattccagcgcaagggtatgggatgatgggatcattgatccagcagacaccagactggtc
ttgggtctcagttttagtgcagccctcaacgcaccaatagagaagactgacttcggtatc
ttcaggatgtaa

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