KEGG   Homo sapiens (human): 56922Help
Entry
56922             CDS       T01001                                 

Gene name
MCCC1, MCC-B, MCCA
Definition
(RefSeq) methylcrotonoyl-CoA carboxylase 1 (EC:6.4.1.4)
  KO
K01968  
3-methylcrotonyl-CoA carboxylase alpha subunit [EC:6.4.1.4]
Organism
hsa  Homo sapiens (human)
Pathway
Valine, leucine and isoleucine degradation
Metabolic pathways
Module
Leucine degradation, leucine => acetoacetate + acetyl-CoA
Disease
H00181  
3-Methylcrotonylglycinuria
Brite
KEGG Orthology (KO) [BR:hsa00001]
 Metabolism
  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)
BRITE hierarchy
SSDB OrthologParalogGFIT
Motif Motif
Other DBs
NCBI-ProteinID: 
NCBI-GeneID: 
OMIM: 
HGNC: 
HPRD: 
Ensembl: 
Vega: 
UniProt: 
Structure
PDB: 

Jmol
Position
3q27
AA seq 725 aa AA seqDB search
MAAASAVSVLLVAAERNRWHRLPSLLLPPRTWVWRQRTMKYTTATGRNITKVLIANRGEI
ACRVMRTAKKLGVQTVAVYSEADRNSMHVDMADEAYSIGPAPSQQSYLSMEKIIQVAKTS
AAQAIHPGCGFLSENMEFAELCKQEGIIFIGPPPSAIRDMGIKSTSKSIMAAAGVPVVEG
YHGEDQSDQCLKEHARRIGYPVMIKAVRGGGGKGMRIVRSEQEFQEQLESARREAKKSFN
DDAMLIEKFVDTPRHVEVQVFGDHHGNAVYLFERDCSVQRRHQKIIEEAPAPGIKSEVRK
KLGEAAVRAAKAVNYVGAGTVEFIMDSKHNFCFMEMNTRLQVEHPVTEMITGTDLVEWQL
RIAAGEKIPLSQEEITLQGHAFEARIYAEDPSNNFMPVAGPLVHLSTPRADPSTRIETGV
RQGDEVSVHYDPMIAKLVVWAADRQAALTKLRYSLRQYNIVGLHTNIDFLLNLSGHPEFE
AGNVHTDFIPQHHKQLLLSRKAAAKESLCQAALGLILKEKAMTDTFTLQAHDQFSPFSSS
SGRRLNISYTRNMTLKDGKNNVAIAVTYNHDGSYSMQIEDKTFQVLGNLYSEGDCTYLKC
SVNGVASKAKLIILENTIYLFSKEGSIEIDIPVPKYLSSVSSQETQGGPLAPMTGTIEKV
FVKAGDKVKAGDSLMVMIAMKMEHTIKSPKDGTVKKVFYREGAQANRHTPLVEFEEEESD
KRESE
NT seq 2178 nt NT seq  +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

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