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 HlyD_D23 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
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

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Ontology (2)   
   KEGG BRITE (2)   
Pathway (3)   
   KEGG PATHWAY (2)   
   KEGG MODULE (1)   
Network (2)   
   KEGG NETWORK (2)   
Disease (3)   
   KEGG DISEASE (1)   
   OMIM (2)   
Chemical substance (6)   
   KEGG COMPOUND (6)   
Chemical reaction (2)   
   KEGG ENZYME (1)   
   KEGG REACTION (1)   
Genome (1)   
   KEGG GENOME (1)   
Gene (12)   
   KEGG ORTHOLOGY (1)   
   RefGene (3)   
   NCBI-PROTEINID (1)   
   NCBI-Gene (1)   
   HGNC (1)   
   Ensembl (1)   
   RIKEN BRC-DNA (3)   
   OC (1)   
Protein sequence (20)   
   UniProt (2)   
   SWISS-PROT (1)   
   RefSeq(pep) (17)   
DNA sequence (65)   
   RefSeq(nuc) (21)   
   GenBank (22)   
   EMBL (22)   
3D Structure (1)   
   PDB (1)   
Protein domain (17)   
   Pfam (17)   
All databases (134)   

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