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NameMethylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
Synonyms
  • 3-methylcrotonyl-CoA carboxylase 1
  • 3-methylcrotonyl-CoA carboxylase biotin-containing subunit
  • 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha
  • 6.4.1.4
  • MCCA
  • MCCase subunit alpha
Gene NameMCCC1
OrganismHuman
Amino acid sequence
>lcl|BSEQ0000887|Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
MAAASAVSVLLVAAERNRWHRLPSLLLPPRTWVWRQRTMKYTTATGRNITKVLIANRGEI
ACRVMRTAKKLGVQTVAVYSEADRNSMHVDMADEAYSIGPAPSQQSYLSMEKIIQVAKTS
AAQAIHPGCGFLSENMEFAELCKQEGIIFIGPPPSAIRDMGIKSTSKSIMAAAGVPVVEG
YHGEDQSDQCLKEHARRIGYPVMIKAVRGGGGKGMRIVRSEQEFQEQLESARREAKKSFN
DDAMLIEKFVDTPRHVEVQVFGDHHGNAVYLFERDCSVQRRHQKIIEEAPAPGIKSEVRK
KLGEAAVRAAKAVNYVGAGTVEFIMDSKHNFCFMEMNTRLQVEHPVTEMITGTDLVEWQL
RIAAGEKIPLSQEEITLQGHAFEARIYAEDPSNNFMPVAGPLVHLSTPRADPSTRIETGV
RQGDEVSVHYDPMIAKLVVWAADRQAALTKLRYSLRQYNIVGLHTNIDFLLNLSGHPEFE
AGNVHTDFIPQHHKQLLLSRKAAAKESLCQAALGLILKEKAMTDTFTLQAHDQFSPFSSS
SGRRLNISYTRNMTLKDGKNNVAIAVTYNHDGSYSMQIEDKTFQVLGNLYSEGDCTYLKC
SVNGVASKAKLIILENTIYLFSKEGSIEIDIPVPKYLSSVSSQETQGGPLAPMTGTIEKV
FVKAGDKVKAGDSLMVMIAMKMEHTIKSPKDGTVKKVFYREGAQANRHTPLVEFEEEESD
KRESE
Number of residues725
Molecular Weight80472.45
Theoretical pI7.86
GO Classification
Functions
  • methylcrotonoyl-CoA carboxylase activity
  • biotin carboxylase activity
  • ATP binding
  • biotin binding
  • metal ion binding
Processes
  • small molecule metabolic process
  • biotin metabolic process
  • branched-chain amino acid catabolic process
  • leucine catabolic process
  • vitamin metabolic process
  • water-soluble vitamin metabolic process
  • cellular nitrogen compound metabolic process
Components
  • cytosol
  • mitochondrion
  • mitochondrial inner membrane
  • mitochondrial matrix
General FunctionMethylcrotonoyl-coa carboxylase activity
Specific FunctionBiotin-attachment subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism.
Pfam Domain Function
Transmembrane RegionsNot Available
GenBank Protein ID12382296
UniProtKB IDQ96RQ3
UniProtKB Entry NameMCCA_HUMAN
Cellular LocationMitochondrion matrix
Gene sequence
>lcl|BSEQ0010310|Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (MCCC1)
ATGGGTGTGGAGGCAAAGAACCATGAAGTACACAACAGCCACAGGCAGATGAAGCATATT
CCATCGGCCCCGCTCCCTCCCAGCAGAGCTACCTATCTATGGAGAAAATCATTCAAGTGG
CCAAGACCTCTGCTGCACAGCACATCCAAATCCATAATGGCTGCTGCTGGAGTACCTGTT
GTGGAGGGTTATCATGGTGAGGACCAATCAGACCAGTGCCTGAAGGAACACGCCAGGAGA
ATTGGCTATCCTGTCATGATTAAAGCCGTCCGGGGTGGAGGAGGAAAAGGAATGAGGATT
GTTAGATCAGAACAAGAATTTCAAGAACAGTTAGAGTCAGCACGGAGAGAAGCTAAGAAG
TCTTTCAATGATGATGCTATGCTGATCGAGAAGTTTGTAGACACACCGAGGCATGTAGAA
GTCCAGGTGTTTGGTGATCACCATGGCAATGCTGTGTACTTGTTTGAAAGAGACTGTAGT
GTGCAGAGGCGACATCAGAAGATCATTGAGGAGGCCCCAGCGCCTGGTATTAAATCTGAA
GTAAGAAAAAAGCTGGGAGAAGCTGCAGTCAGAGCTGCTAAAGCTGTAAATTATGTTGGA
GCAGGGACTGTGGAGTTTATTATGGACTCAAAACATAATTTCTGTTTCATGGAGATGAAT
ACAAGGCTGCAAGTGGAACATCCTGTTACTGAGATGATCACAGGAACTGACTTGGTGGAG
TGGCAGCTTAGAATTGCAGCAGGAGAGAAGATTCCTTTGAGCCAGGAAGAAATAACTCTG
CAGGGCCATGCCTTCGAAGCTAGAATATATGCAGAAGATCCTAGCAATAACTTCATGCCT
GTGGCAGGCCCATTAGTGCACCTCTCTACTCCTCGAGCAGACCCTTCCACCAGGATTGAA
ACTGGAGTACGGCAAGGAGACGAAGTTTCCGTGCATTATGACCCCATGATTGCGAAGCTG
GTCGTGTGGGCAGCAGATCGCCAGGCGGCATTGACAAAACTGAGGTACAGCCTTCGTCAG
TACAATATTGTTGGACTGCACACCAACATTGACTTCTTACTCAACCTGTCTGGCCACCCA
GAGTTTGAAGCTGGGAACGTGCACACTGATTTCATCCCTCAACACCACAAACAGTTGTTG
CTCAGTCGGAAGGCTGCAGCCAAAGAGTCTTTATGCCAGGCAGCCCTGGGTCTCATCCTC
AAGGAGAAAGCCATGACCGACACTTTCACTCTTCAGGCACATGATCAATTCTCTCCATTT
TCGTCTAGCAGTGGAAGAAGACTGAATATCTCGTATACCAGAAACATGACTCTTAAAGAT
GGTAAAAACAATGTAGCCATAGCTGTAACGTATAACCATGATGGGTCTTATAGCATGCAG
ATTGAAGATAAAACTTTCCAAGTCCTTGGTAATCTTTACAGCGAGGGAGACTGCACTTAC
CTGAAATGTTCTGTTAATGGAGTTGCTAGTAAAGCGAAGCTGATTATCCTGGAAAACACT
ATTTACCTATTTTCCAAGGAAGGAAGTATTGAGATTGACATTCCAGTCCCCAAATACTTA
TCTTCTGTGAGCTCACAAGAAACTCAGGGCGGCCCCTTAGCTCCTATGACTGGAACCATT
GAAAAGGTGTTTGTCAAAGCTGGAGACAAAGTGAAAGCGGGAGATTCCCTCATGGTTATG
ATCGCCATGAAGATGGAGCATACCATAAAGTCTCCAAAGGATGGCACAGTAAAGAAAGTG
TTCTACAGAGAAGGTGCTCAGGCCAACAGACACACTCCTTTAGTCGAGTTTGAGGAGGAA
GAATCAGACAAAAGGGAATCGGAATAA
GenBank Gene IDAF310972
GeneCard IDNot Available
GenAtlas IDMCCC1
HGNC IDHGNC:6936
Chromosome Location3
Locus3q27
References
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  3. Holzinger A, Roschinger W, Lagler F, Mayerhofer PU, Lichtner P, Kattenfeld T, Thuy LP, Nyhan WL, Koch HG, Muntau AC, Roscher AA: Cloning of the human MCCA and MCCB genes and mutations therein reveal the molecular cause of 3-methylcrotonyl-CoA: carboxylase deficiency. Hum Mol Genet. 2001 Jun 1;10(12):1299-306. 11406611
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