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FLASH GENE
Symbol MCCC2 contributors: - updated : 09-03-2007
HGNC name methylcrotonoyl-Coenzyme A carboxylase 2 (beta)
HGNC id 6937
Corresponding disease
MCCC2 3-@methylcrotonyl-CoA carboxylase 2 deficiency 2
Location 5q12-q13      Physical location : 70.883.114 - 70.954.528
Synonym name
  • biotin carboxylase
  • 3- Methylcrotonyl-CoA carboxylase non-biotin-containing subunit
  • MCCase subunit beta
  • Synonym symbol(s) MCCB, MCCD2
    EC.number 6.4.1.4
    DNA
    TYPE functioning gene
    STRUCTURE 70.00 kb     17 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status provisional
    Map cen - D5S427 - D5S2072 - D5S637 /MCCC2 MCCC2 - D5S650 - D5S2041 - qter
    Authors Gallardo (01)
    Physical map
    KENAE 5q13.1 Kenae LOC285653 5q13.1 similar to chromosome 7 open reading frame 17 protein; 16.7kD protein TAF9 5q12 TAF9 RNA polymerase II, TATA box binding protein (TBP)-associated factor, 32kDa RAD17 5q12-q13.1 RAD17 homolog (S. pombe) FLJ30532 5q13.1 hypothetical protein FLJ30532 OCLN 5q13.1 occludin GTF2H2 5q13.1 general transcription factor IIH, polypeptide 2, 44kDa BIRC1 5q13.1 baculoviral IAP repeat-containing 1 SMN1 5q13.1 survival of motor neuron 1, telomeric LOC391791 5 similar to cadherin 12, type 2 preproprotein; Br-cadherin; cadherin-12; N-cadherin 2; brain-cadherin; neuronal cadherin 2; cadherin, neural type, 2 SMA3 5q13 SMA3 LOC389294 5 similar to bA110H4.2 (similar to membrane protein) LOC391792 5 similar to SMA3 protein LOC340089 5q13.2 similar to bA110H4.2 (similar to membrane protein) LOC391793 5 similar to cadherin 12, type 2 preproprotein; Br-cadherin; cadherin-12; N-cadherin 2; brain-cadherin; neuronal cadherin 2; cadherin, neural type, 2 SERF1A 5q13.1 small EDRK-rich factor 1A (telomeric) SMN2 5q13.1 survival of motor neuron 2, centromeric LOC389295 5 LOC389295 LOC389296 5 similar to Beta-glucuronidase precursor (Beta-G1) LOC389297 5 similar to bA110H4.2 (similar to membrane protein) LOC389298 5 similar to bA110H4.2 (similar to membrane protein) LOC389299 5 similar to Beta-glucuronidase precursor (Beta-G1) LOC391794 5 similar to cadherin 12, type 2 preproprotein; Br-cadherin; cadherin-12; N-cadherin 2; brain-cadherin; neuronal cadherin 2; cadherin, neural type, 2 PMCHL2 5q13 pro-melanin-concentrating hormone-like 2 BDP1 5q13 B double prime 1, subunit of RNA polymerase III transcription initiation factor IIIB MCCC2 5q12-q13 methylcrotonoyl-Coenzyme A carboxylase 2 (beta) CART 5q13.1 Homo sapiens cocaine- and amphetamine-regulated transcript (CART), mRNA. MAP1B 5q13 microtubule-associated protein 1B MRPS27 5q13.2 mitochondrial ribosomal protein S27 FLJ12598 5q13.2 hypothetical protein FLJ12598 LOC391795 5 similar to ribosomal protein L10a ZNF366 5q13.1 zinc finger protein 366 LOC389300 5 LOC389300 KPNB2 5p14.2-q22.3 karyopherin (importin) beta 2 LOC115548 5q13.2 hypothetical protein BC014311 LOC134285 5q13.3 hypothetical protein LOC134285 MGC13034 5q13.3 hypothetical protein MGC13034 LOC389301 5 similar to nonhistone chromosomal protein HMG-17 - rat LOC389302 5 LOC389302 FOXD1 5q12-q13 forkhead box D1 BTF3 5p13.2 basic transcription factor 3 LOC220793 5q13.3 similar to hepatitis C virus core-binding protein 6; cervical cancer oncogene 3 ANKRA2 5q12-q13 ankyrin repeat, family A (RFXANK-like), 2 FLJ12787 5q13.3 hypothetical protein FLJ12787 PRO1942 5q13.3 hypothetical protein PRO1942 RGNEF 5q13.3 Rho-guanine nucleotide exchange factor
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    - - 4000 - - - Gallardo
    - polyA site 2400 613 563 - Gallardo
    EXPRESSION
    Type
    constitutive of
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Cardiovascularheart   highly
    Digestiveliver   highly
    Urinarykidney   highly
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Muscularstriatumcardiac  
    Muscularstriatumskeletal  
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • carboxyltransferase domain
  • a binding site for the acyl-Coa substrate
  • putative signal peptide
  • secondary structure alpha (biotin containing) and beta subunit
    mono polymer heteromer , dimer
    HOMOLOGY
    interspecies ortholog to Mccb aspergillus midulary
    ortholog to rattus mccc2
    Homologene
    FAMILY accD/PCCB family
    CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,inner
    basic FUNCTION
  • catalyzing carboxylation of 3 methylcrotonyl COA to form 3 methylglutaconyl-COA
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism aminoacid
    signaling
    leucine catabolism
    a component
    INTERACTION
    DNA
    RNA
    small molecule metal binding,
    magnesium Mg2+
    protein
    cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) MCCC2
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS