KEGG   ORTHOLOGY: K08900Help
Entry
K08900                      KO                                     

Name
BCS1
Definition
mitochondrial chaperone BCS1
Disease
H00820  Bjornstad syndrome
H01354  Leigh syndrome
H02007  GRACILE syndrome
H02086  Mitochondrial complex III deficiency
Brite
KEGG Orthology (KO) [BR:ko00001]
 09180 Brite Hierarchies
  09182 Protein families: genetic information processing
   03029 Mitochondrial biogenesis
    K08900  BCS1; mitochondrial chaperone BCS1
Mitochondrial biogenesis [BR:ko03029]
 Mitochondrial quality control factors
  Mitochondrial respiratory chain complex assembly factors
   Complex-III assembly factors
    K08900  BCS1; mitochondrial chaperone BCS1
BRITE hierarchy
Genes
HSA: 617(BCS1L)
PTR: 459946(BCS1L)
PPS: 100988698(BCS1L)
GGO: 101129851
PON: 100437347
NLE: 100604478(BCS1L)
MCC: 699690(BCS1L)
MCF: 102123539
CSAB: 103217874(BCS1L)
RRO: 104660028
RBB: 108518458
CJC: 100390879(BCS1L)
SBQ: 101030917
MMU: 66821(Bcs1l)
MCAL: 110304618
MPAH: 110321210
RNO: 301514(Bcs1l)
MUN: 110566690
CGE: 100771607
NGI: 103727722
HGL: 101703804
CCAN: 109681369
OCU: 100351828(BCS1L)
TUP: 102474109
CFA: 478911
VVP: 112922551
AML: 100480908(BCS1L)
UMR: 103662759
UAH: 113250450
ORO: 101372781
FCA: 101090709
PTG: 102957048
AJU: 106970107
BTA: 539713(BCS1L)
BOM: 102274064
BIU: 109570660
BBUB: 102395153
PHD: 102328698
CHX: 102169634
OAS: 101111607
SSC: 100626589(BCS1L)
CFR: 102516038
CDK: 105096086
BACU: 103019425
OOR: 101289848
DLE: 111171833
PCAD: 102975790
ECB: 100055811(BCS1L)
EPZ: 103561960
EAI: 106838454
MYB: 102254268
MYD: 102759665
MNA: 107524850
HAI: 109380564
DRO: 112307980
PALE: 102884217
RAY: 107516724
LAV: 100663108
TMU: 101346481
MDO: 100011174(BCS1L)
SHR: 100919063
PCW: 110219454
OAA: 100088885
GGA: 424212(BCS1L)
MGP: 100539820
CJO: 107316916
NMEL: 110400533
APLA: 101804030
ACYG: 106039323
TGU: 100217866
LSR: 110468346
SCAN: 103814353
GFR: 102035159
FAB: 101818392
PHI: 102100470
PMAJ: 107207603
CCAE: 111932188
CCW: 104693969
ETL: 114055309
FPG: 101920870
FCH: 102054232
CLV: 102084072
EGZ: 104132407
NNI: 104010070
ACUN: 113482394
PADL: 103917660
AAM: 106499293
ASN: 102386059
AMJ: 102570697(BCS1L) 106737726
PSS: 102458646
CMY: 102934334
CPIC: 101950145
ACS: 100552242
PVT: 110078237
PBI: 103059046
PMUR: 107291479
GJA: 107116965
XLA: 108701304 380366(bcs1l.L)
XTR: 549619(bcs1l)
NPR: 108783879
DRE: 394157(bcs1l)
SANH: 107673730
IPU: 108266488
PHYP: 113525793
AMEX: 103024599
EEE: 113577203
TRU: 101062889
LCO: 104936008
MZE: 101465291
OLA: 101159054
XMA: 102234420
XCO: 114147795
PRET: 103461861
NFU: 107396454
KMR: 108244286
AOCE: 111568523
CSEM: 103380769
POV: 109637352
LCF: 108897206
SDU: 111218464
HCQ: 109517562
BPEC: 110171371
MALB: 109972625
SASA: 106581856(BCS1)
OTW: 112225126
SALP: 111959728
ELS: 105016180
SFM: 108924223
PKI: 111845386
LCM: 102353356
CMK: 103189807
CIN: 100181911
SPU: 579223
APLC: 110983248
SKO: 100373593(Bcs1l)
DME: Dmel_CG4908(CG4908)
DER: 6543296
DPE: 6589332
DSI: Dsimw501_GD23665(Dsim_GD23665)
DWI: 26528938
DAZ: 108611976
DNV: 108650013
DHE: 111596641
MDE: 101895172
LCQ: 111685091
AAG: 5574412
AME: 552253
BIM: 100743520
BTER: 100650655
SOC: 105203992
MPHA: 105834075
AEC: 105149548
ACEP: 105622394
PBAR: 105429900
VEM: 105562905
HST: 105188058
DQU: 106743898
CFO: 105249499
LHU: 105669594
PGC: 109863623
OBO: 105276498
PCF: 106787652
MDL: 103579753
TCA: 660477
DPA: 109534243
NVL: 108569424
BMOR: 101736280
PMAC: 106712330
PRAP: 111003140
HAW: 110371416
TNL: 113492660
PXY: 105398371
CLEC: 106669641
ZNE: 110834516
DPTE: 113795161
CEL: CELE_F54C9.6(bcs-1)
CBR: CBG00560
BMY: Bm1_30135
PCAN: 112570803
CRG: 105345623
MYI: 110442582
OBI: 106871007
LAK: 106166292
SHX: MS3_09362
EGL: EGR_04258
EPA: 110235005
PDAM: 113684820
SPIS: 111322625
HMG: 100208803
AQU: 100639781
LJA: Lj1g3v1032230.1(Lj1g3v1032230.1) Lj1g3v2940710.1(Lj1g3v2940710.1) Lj1g3v2941740.1(Lj1g3v2941740.1) Lj2g3v1024100.1(Lj2g3v1024100.1) Lj2g3v1024120.1(Lj2g3v1024120.1) Lj2g3v1024160.1(Lj2g3v1024160.1) Lj2g3v1705510.1(Lj2g3v1705510.1) Lj2g3v1729010.1(Lj2g3v1729010.1) Lj2g3v1729020.1(Lj2g3v1729020.1) Lj2g3v1729190.1(Lj2g3v1729190.1) Lj3g3v0933420.1(Lj3g3v0933420.1) Lj3g3v1061340.1(Lj3g3v1061340.1) Lj3g3v1061350.1(Lj3g3v1061350.1) Lj3g3v2478860.1(Lj3g3v2478860.1) Lj4g3v0548780.1(Lj4g3v0548780.1) Lj5g3v2300310.1(Lj5g3v2300310.1) Lj6g3v1527000.1(Lj6g3v1527000.1)
OSA: 4339775
DOSA: Os03t0584400-01(Os03g0584400) Os05t0588900-01(Os05g0588900) Os07t0517800-00(Os07g0517800) Os12t0470700-01(Os12g0470700) Os12t0639400-01(Os12g0639400)
ATS: 109738210(LOC109738210) 109739261(LOC109739261) 109739802(LOC109739802) 109742675(LOC109742675) 109742676(LOC109742676) 109742677(LOC109742677) 109742687(LOC109742687) 109742688(LOC109742688) 109747259(LOC109747259) 109754240(LOC109754240) 109771668(LOC109771668) 109775090(LOC109775090)
CRE: CHLREDRAFT_179044(BCS1)
MNG: MNEG_0052
SCE: YDR375C(BCS1)
ERC: Ecym_4643
NCS: NCAS_0H02120(NCAS0H02120)
NDI: NDAI_0C01530(NDAI0C01530)
TPF: TPHA_0E01660(TPHA0E01660)
TBL: TBLA_0A02720(TBLA0A02720)
TDL: TDEL_0D02530(TDEL0D02530)
KAF: KAFR_0D05090(KAFR0D05090)
CAL: CAALFM_CR05970CA(CaO19.458)
SLB: AWJ20_5248(BCS1)
NTE: NEUTE1DRAFT124799(NEUTE1DRAFT_124799) NEUTE1DRAFT72346(NEUTE1DRAFT_72346)
ANG: ANI_1_1282024(An02g08900) ANI_1_1372134(An15g03540)
CNE: CNC04110
CNB: CNBC3080
ABP: AGABI1DRAFT108111(AGABI1DRAFT_108111) AGABI1DRAFT108687(AGABI1DRAFT_108687) AGABI1DRAFT116485(AGABI1DRAFT_116485) AGABI1DRAFT34350(AGABI1DRAFT_34350) AGABI1DRAFT36444(AGABI1DRAFT_36444) AGABI1DRAFT62968(AGABI1DRAFT_62968)
ABV: AGABI2DRAFT180594(AGABI2DRAFT_180594) AGABI2DRAFT180828(AGABI2DRAFT_180828) AGABI2DRAFT196029(AGABI2DRAFT_196029) AGABI2DRAFT210088(AGABI2DRAFT_210088) AGABI2DRAFT63145(AGABI2DRAFT_63145) AGABI2DRAFT70517(AGABI2DRAFT_70517)
MGL: MGL_3700
DDI: DDB_G0286765 DDB_G0289135(bcs1lA) DDB_G0291910(bcs1lB)
DFA: DFA_01976(bcs1lB) DFA_03149 DFA_03225(bcs1lA)
PYO: PY17X_0103600(PY02578)
PCB: PCHAS_010270(PC000268.03.0)
SMIN: v1.2.003867.t1(symbB.v1.2.003867.t1) v1.2.003868.t1(symbB.v1.2.003868.t1) v1.2.026380.t1(symbB.v1.2.026380.t1)
SPAR: SPRG_09317
DFC: DFI_01405
PBOR: BSF38_05768(yjoB)
 » show all
TaxonomyKoalaUniProt
Reference
  Authors
Blazquez A, Gil-Borlado MC, Moran M, Verdu A, Cazorla-Calleja MR, Martin MA, Arenas J, Ugalde C
  Title
Infantile mitochondrial encephalomyopathy with unusual phenotype caused by a novel BCS1L mutation in an isolated complex III-deficient patient.
  Journal
Neuromuscul Disord 19:143-6 (2009)
DOI:10.1016/j.nmd.2008.11.016
  Sequence
[hsa:617]
Reference
  Authors
Tamai S, Iida H, Yokota S, Sayano T, Kiguchiya S, Ishihara N, Hayashi J, Mihara K, Oka T
  Title
Characterization of the mitochondrial protein LETM1, which maintains the mitochondrial tubular shapes and interacts with the AAA-ATPase BCS1L.
  Journal
J Cell Sci 121:2588-600 (2008)
DOI:10.1242/jcs.026625
  Sequence
[hsa:617]

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