Details for: BCS1L

Gene ID: 617

Symbol: BCS1L

Ensembl ID: ENSG00000074582

Description: BCS1 homolog, ubiquinol-cytochrome c reductase complex chaperone

Associated with

Other Information

Genular Protein ID: 3336524564

Symbol: BCS1_HUMAN

Name: Mitochondrial chaperone BCS1

UniProtKB Accession Codes:

Database IDs:

Citations:

PubMed ID: 9878253

Title: Identification and characterization of human cDNAs specific to BCS1, PET112, SCO1, COX15, and COX11, five genes involved in the formation and function of the mitochondrial respiratory chain.

PubMed ID: 9878253

DOI: 10.1006/geno.1998.5580

PubMed ID: 11528392

Title: A mutant mitochondrial respiratory chain assembly protein causes complex III deficiency in patients with tubulopathy, encephalopathy and liver failure.

PubMed ID: 11528392

DOI: 10.1038/ng706

PubMed ID: 12215968

Title: GRACILE syndrome, a lethal metabolic disorder with iron overload, is caused by a point mutation in BCS1L.

PubMed ID: 12215968

DOI: 10.1086/342773

PubMed ID: 9110174

Title: Large-scale concatenation cDNA sequencing.

PubMed ID: 9110174

DOI: 10.1101/gr.7.4.353

PubMed ID: 14702039

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

PubMed ID: 14702039

DOI: 10.1038/ng1285

PubMed ID: 17974005

Title: The full-ORF clone resource of the German cDNA consortium.

PubMed ID: 17974005

DOI: 10.1186/1471-2164-8-399

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: 18628306

Title: Characterization of the mitochondrial protein LETM1, which maintains the mitochondrial tubular shapes and interacts with the AAA-ATPase BCS1L.

PubMed ID: 18628306

DOI: 10.1242/jcs.026625

PubMed ID: 21269460

Title: Initial characterization of the human central proteome.

PubMed ID: 21269460

DOI: 10.1186/1752-0509-5-17

PubMed ID: 23186163

Title: Toward a comprehensive characterization of a human cancer cell phosphoproteome.

PubMed ID: 23186163

DOI: 10.1021/pr300630k

PubMed ID: 25944712

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

PubMed ID: 25944712

DOI: 10.1002/pmic.201400617

PubMed ID: 12910490

Title: Clinical and diagnostic characteristics of complex III deficiency due to mutations in the BCS1L gene.

PubMed ID: 12910490

DOI: 10.1002/ajmg.a.20171

PubMed ID: 17403714

Title: Impaired complex III assembly associated with BCS1L gene mutations in isolated mitochondrial encephalopathy.

PubMed ID: 17403714

DOI: 10.1093/hmg/ddm072

PubMed ID: 17314340

Title: Missense mutations in the BCS1L gene as a cause of the Bjoernstad syndrome.

PubMed ID: 17314340

DOI: 10.1056/nejmoa055262

PubMed ID: 19162478

Title: Infantile mitochondrial encephalomyopathy with unusual phenotype caused by a novel BCS1L mutation in an isolated complex III-deficient patient.

PubMed ID: 19162478

DOI: 10.1016/j.nmd.2008.11.016

PubMed ID: 24172246

Title: Novel mutation in AAA domain of BCS1L causing Bjornstad syndrome.

PubMed ID: 24172246

DOI: 10.1038/jhg.2013.101

PubMed ID: 22991165

Title: Clinical and biochemical features associated with BCS1L mutation.

PubMed ID: 22991165

DOI: 10.1007/s10545-012-9536-4

Sequence Information:

  • Length: 419
  • Mass: 47534
  • Checksum: 7F0F98BA62F2CBB8
  • Sequence:
  • MPLSDFILAL KDNPYFGAGF GLVGVGTALA LARKGVQLGL VAFRRHYMIT LEVPARDRSY 
    AWLLSWLTRH STRTQHLSVE TSYLQHESGR ISTKFEFVPS PGNHFIWYRG KWIRVERSRE 
    MQMIDLQTGT PWESVTFTAL GTDRKVFFNI LEEARELALQ QEEGKTVMYT AVGSEWRPFG 
    YPRRRRPLNS VVLQQGLADR IVRDVQEFID NPKWYTDRGI PYRRGYLLYG PPGCGKSSFI 
    TALAGELEHS ICLLSLTDSS LSDDRLNHLL SVAPQQSLVL LEDVDAAFLS RDLAVENPVK 
    YQGLGRLTFS GLLNALDGVA STEARIVFMT TNHVDRLDPA LIRPGRVDLK EYVGYCSHWQ 
    LTQMFQRFYP GQAPSLAENF AEHVLRATNQ ISPAQVQGYF MLYKNDPVGA IHNAESLRR

Genular Protein ID: 2908627592

Symbol: A8JZZ8_HUMAN

Name: N/A

UniProtKB Accession Codes:

Database IDs:

Sequence Information:

  • Length: 419
  • Mass: 47550
  • Checksum: 2E4BD69C0EF9EBFF
  • Sequence:
  • MPLSDFILAL KDNPYFGAGF GLVGVGTALA LARKGVQLGL VAFRRHYMIT LEVPARDRSY 
    AWLLSWLTRH STRTQHLSVE TSYLQHESGR ISTKFEFVPS LGNHFIWYRG KWIRVERSRE 
    MQMIDLQTGT PWESVTFTAL GTDRKVFFNI LEEARELALQ QEEGKTVMYT AVGSEWRPFG 
    YPRRRRPLNS VVLQQGLADR IVRDVQEFID NPKWYTDRGI PYRRGYLLYG PPGCGKSSFI 
    TALAGELEHS ICLLSLTDSS LSDDRLNHLL SVAPQQSLVL LEDVDAAFLS RDLAVENPVK 
    YQGLGRLTFS GLLNALDGVA STEARIVFMT TNHVDRLDPA LIRPGRVDLK EYVGYCSHWQ 
    LTQMFQRFYP GQAPSLAENF AEHVLRATNQ ISPAQVQGYF MLYKNDPVGA IHNAESLRR

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.