Details for: MCCC1

Gene ID: 56922

Symbol: MCCC1

Ensembl ID: ENSG00000078070

Description: methylcrotonyl-CoA carboxylase subunit 1

Associated with

Other Information

Genular Protein ID: 3467690564

Symbol: MCCA_HUMAN

Name: Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial

UniProtKB Accession Codes:

Database IDs:

Citations:

PubMed ID: 11170888

Title: The molecular basis of 3-methylcrotonylglycinuria, a disorder of leucine catabolism.

PubMed ID: 11170888

DOI: 10.1086/318202

PubMed ID: 11401427

Title: Human biotin-containing subunit of 3-methylcrotonyl-CoA carboxylase gene (MCCA): cDNA sequence, genomic organization, localization to chromosomal band 3q27, and expression.

PubMed ID: 11401427

DOI: 10.1006/geno.2000.6366

PubMed ID: 11406611

Title: Cloning of the human MCCA and MCCB genes and mutations therein reveal the molecular cause of 3-methylcrotonyl-CoA: carboxylase deficiency.

PubMed ID: 11406611

DOI: 10.1093/hmg/10.12.1299

PubMed ID: 11181649

Title: The molecular basis of human 3-methylcrotonyl-CoA carboxylase deficiency.

PubMed ID: 11181649

DOI: 10.1172/jci11948

PubMed ID: 14702039

Title: Complete sequencing and characterization of 21,243 full-length human cDNAs.

PubMed ID: 14702039

DOI: 10.1038/ng1285

PubMed ID: 15489334

Title: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).

PubMed ID: 15489334

DOI: 10.1101/gr.2596504

PubMed ID: 16023992

Title: Mitochondrial targeting signals and mature peptides of 3-methylcrotonyl-CoA carboxylase.

PubMed ID: 16023992

DOI: 10.1016/j.bbrc.2005.06.190

PubMed ID: 17360195

Title: Expression, purification, characterization of human 3-methylcrotonyl-CoA carboxylase (MCCC).

PubMed ID: 17360195

DOI: 10.1016/j.pep.2007.01.012

PubMed ID: 21269460

Title: Initial characterization of the human central proteome.

PubMed ID: 21269460

DOI: 10.1186/1752-0509-5-17

PubMed ID: 23438705

Title: Mitochondrial SIRT4-type proteins in Caenorhabditis elegans and mammals interact with pyruvate carboxylase and other acetylated biotin-dependent carboxylases.

PubMed ID: 23438705

DOI: 10.1016/j.mito.2013.02.002

PubMed ID: 24275569

Title: An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome.

PubMed ID: 24275569

DOI: 10.1016/j.jprot.2013.11.014

PubMed ID: 25944712

Title: N-terminome analysis of the human mitochondrial proteome.

PubMed ID: 25944712

DOI: 10.1002/pmic.201400617

PubMed ID: 32561715

Title: Leucine regulates autophagy via acetylation of the mTORC1 component raptor.

PubMed ID: 32561715

DOI: 10.1038/s41467-020-16886-2

PubMed ID: 16010683

Title: 3-Methylcrotonyl-CoA carboxylase deficiency: mutation analysis in 28 probands, 9 symptomatic and 19 detected by newborn screening.

PubMed ID: 16010683

DOI: 10.1002/humu.9352

PubMed ID: 17968484

Title: Novel mutations in five Japanese patients with 3-methylcrotonyl-CoA carboxylase deficiency.

PubMed ID: 17968484

DOI: 10.1007/s10038-007-0211-9

PubMed ID: 21071250

Title: Novel mutations in the human MCCA and MCCB gene causing methylcrotonylglycinuria.

PubMed ID: 21071250

DOI: 10.1016/j.ymgme.2010.10.008

PubMed ID: 22150417

Title: Mutational spectrum in eight Korean patients with 3-methylcrotonyl-CoA carboxylase deficiency.

PubMed ID: 22150417

DOI: 10.1111/j.1399-0004.2011.01704.x

PubMed ID: 22264772

Title: A single mutation in MCCC1 or MCCC2 as a potential cause of positive screening for 3-methylcrotonyl-CoA carboxylase deficiency.

PubMed ID: 22264772

DOI: 10.1016/j.ymgme.2011.12.018

PubMed ID: 22642865

Title: 3-methylcrotonyl-CoA carboxylase deficiency: clinical, biochemical, enzymatic and molecular studies in 88 individuals.

PubMed ID: 22642865

DOI: 10.1186/1750-1172-7-31

PubMed ID: 25382614

Title: Identification of eight novel mutations and transcript analysis of two splicing mutations in Chinese newborns with MCC deficiency.

PubMed ID: 25382614

DOI: 10.1111/cge.12535

PubMed ID: 27601257

Title: 3-Methylcrotonyl-CoA carboxylase deficiency: Mutational spectrum derived from comprehensive newborn screening.

PubMed ID: 27601257

DOI: 10.1016/j.gene.2016.09.003

PubMed ID: 28887846

Title: Mutations in KARS cause early-onset hearing loss and leukoencephalopathy: Potential pathogenic mechanism.

PubMed ID: 28887846

DOI: 10.1002/humu.23335

Sequence Information:

  • Length: 725
  • Mass: 80473
  • Checksum: B84AD23806035A40
  • Sequence:
  • MAAASAVSVL LVAAERNRWH RLPSLLLPPR TWVWRQRTMK YTTATGRNIT KVLIANRGEI 
    ACRVMRTAKK LGVQTVAVYS EADRNSMHVD MADEAYSIGP APSQQSYLSM EKIIQVAKTS 
    AAQAIHPGCG FLSENMEFAE LCKQEGIIFI GPPPSAIRDM GIKSTSKSIM AAAGVPVVEG 
    YHGEDQSDQC LKEHARRIGY PVMIKAVRGG GGKGMRIVRS EQEFQEQLES ARREAKKSFN 
    DDAMLIEKFV DTPRHVEVQV FGDHHGNAVY LFERDCSVQR RHQKIIEEAP APGIKSEVRK 
    KLGEAAVRAA KAVNYVGAGT VEFIMDSKHN FCFMEMNTRL QVEHPVTEMI TGTDLVEWQL 
    RIAAGEKIPL SQEEITLQGH AFEARIYAED PSNNFMPVAG PLVHLSTPRA DPSTRIETGV 
    RQGDEVSVHY DPMIAKLVVW AADRQAALTK LRYSLRQYNI VGLHTNIDFL LNLSGHPEFE 
    AGNVHTDFIP QHHKQLLLSR KAAAKESLCQ AALGLILKEK AMTDTFTLQA HDQFSPFSSS 
    SGRRLNISYT RNMTLKDGKN NVAIAVTYNH DGSYSMQIED KTFQVLGNLY SEGDCTYLKC 
    SVNGVASKAK LIILENTIYL FSKEGSIEID IPVPKYLSSV SSQETQGGPL APMTGTIEKV 
    FVKAGDKVKA GDSLMVMIAM KMEHTIKSPK DGTVKKVFYR EGAQANRHTP LVEFEEEESD 
    KRESE

Genular Protein ID: 3309952262

Symbol: Q68D27_HUMAN

Name: N/A

UniProtKB Accession Codes:

Database IDs:

Sequence Information:

  • Length: 575
  • Mass: 63967
  • Checksum: 230DF52EBB05AAE9
  • Sequence:
  • MEKIIQVAKT SAAQHIQSIM AAAGVPVVEG YHGEDQSDQC LKEHARRIGY PVMIKAVRGG 
    GGKGMRIVRS EQEFQEQLES ARREAKKSFN DDAMLIEKFV DTPRHVEVQV FGDHHGNAVY 
    LFERDCSVQR RHQKIIEEAP APGIKSEVRK KLGEAAVRAA KAVNYVGAGT VEFIMDSKHN 
    FCFMEMNTRL QVEHPVTEMI TGTDLVEWQL RIAAGEKIPL SQEEITLQGH AFEARIYAED 
    PSNNFMPVAG PLVHLSTPRA DPSTRIETGV RQGDEVSVHY DPMIAKLVVW AADRQAALTK 
    LRYSLRQYNI VGLPTNIDFL LNLSGHPEFE AGNVHTDFIP QHHKQLLLSR KAAAKESLCQ 
    AALGLILKEK AMTDTFTLQA HDQFSPFSSS SGRRLNISYT RNMTLKDGKN NVAIAVTYNH 
    DGSYSMQIED KTFQVLGNLY SEGDCTYLKC SVNGVASKAK LIILENTIYL FSKEGSIEID 
    IPVPKYLSSV SSQETQGGPL APMTGTIEKV FVKAGDKVKA GDSLMVMIAM KMEHTIKSPK 
    DGTVKKVFYR EGAQANRHTP LVEFEEEESD KRESE

Database document:

This is a preview of the gene's schema. Only a few entries are kept for 'singleCellExpressions,' 'mRNAExpressions,' and other large data arrays for visualization purposes. You can zoom in with the mouse wheel for a closer view, and the text will adjust automatically if necessary. For the full schema, download it here.