KEGG   Homo sapiens (human): 56922
Entry
56922             CDS       T01001                                 
Symbol
MCCC1, MCC-B, MCCA, MCCCalpha
Name
(RefSeq) methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1 precursor
  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 BSH_CusB BSH_YknX RimK ATP-grasp_3 ATPgrasp_ST ATP-grasp_LDMS BSH_RND BT_MCC_alpha ATP-grasp_4 GARS_A RnfC_N NQRA_N BSH_CzcB YqfD 2OG-FeII_Oxy_2 BSH_YhbJ
Other DBs
NCBI-GeneID: 56922
NCBI-ProteinID: NP_064551
OMIM: 609010
HGNC: 6936
Ensembl: ENSP00000265594.4
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

  All links  
Disease (2)   
   OMIM (2)   
Gene (11)   
   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 (14)   
   PDB (14)   
Protein domain (22)   
   Pfam (22)   
All databases (134)   

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