KEGG ORTHOLOGY: K02126

ORTHOLOGY: K02126

Entry

K02126                      KO

Symbol

ATPeF0A, MTATP6, ATP6

Name

F-type H+-transporting ATPase subunit a

Pathway

map00190

Oxidative phosphorylation

map01100

Metabolic pathways

map04714

Thermogenesis

map05010

Alzheimer disease

map05012

Parkinson disease

map05014

Amyotrophic lateral sclerosis

map05016

Huntington disease

map05020

Prion disease

map05022

Pathways of neurodegeneration - multiple diseases

map05208

Chemical carcinogenesis - reactive oxygen species

map05415

Diabetic cardiomyopathy

Module

M00158

F-type ATPase, eukaryotes

Disease

H00068

Leber hereditary optic atrophy

H01177

Infantile bilateral striatal necrosis

H01355

Kearns-Sayre syndrome

H01363

NARP syndrome

H01369

ATP synthase deficiency

Brite

KEGG Orthology (KO) [BR:ko00001]
09100 Metabolism
  09102 Energy metabolism
   00190 Oxidative phosphorylation
    K02126  ATPeF0A, MTATP6, ATP6; F-type H+-transporting ATPase subunit a
09150 Organismal Systems
  09159 Environmental adaptation
   04714 Thermogenesis
    K02126  ATPeF0A, MTATP6, ATP6; F-type H+-transporting ATPase subunit a
09160 Human Diseases
  09161 Cancer: overview
   05208 Chemical carcinogenesis - reactive oxygen species
    K02126  ATPeF0A, MTATP6, ATP6; F-type H+-transporting ATPase subunit a
  09164 Neurodegenerative disease
   05010 Alzheimer disease
    K02126  ATPeF0A, MTATP6, ATP6; F-type H+-transporting ATPase subunit a
   05012 Parkinson disease
    K02126  ATPeF0A, MTATP6, ATP6; F-type H+-transporting ATPase subunit a
   05014 Amyotrophic lateral sclerosis
    K02126  ATPeF0A, MTATP6, ATP6; F-type H+-transporting ATPase subunit a
   05016 Huntington disease
    K02126  ATPeF0A, MTATP6, ATP6; F-type H+-transporting ATPase subunit a
   05020 Prion disease
    K02126  ATPeF0A, MTATP6, ATP6; F-type H+-transporting ATPase subunit a
   05022 Pathways of neurodegeneration - multiple diseases
    K02126  ATPeF0A, MTATP6, ATP6; F-type H+-transporting ATPase subunit a
  09166 Cardiovascular disease
   05415 Diabetic cardiomyopathy
    K02126  ATPeF0A, MTATP6, ATP6; F-type H+-transporting ATPase subunit a
09180 Brite Hierarchies
  09182 Protein families: genetic information processing
   03029 Mitochondrial biogenesis
    K02126  ATPeF0A, MTATP6, ATP6; F-type H+-transporting ATPase subunit a
Mitochondrial biogenesis [BR:ko03029]
Mitochondrial DNA transcription, translation, and replication factors
  Mitochondrial DNA-encoded proteins
   F-type ATPase
    K02126  ATPeF0A, MTATP6, ATP6; F-type H+-transporting ATPase subunit a

Other DBs

COG:

COG0356

TC:

3.A.2.1

Genes

HSA:	4508(ATP6)

PTR:	807859(ATP6)

PPS:	807880(ATP6)

GGO:	6742688(ATP6)

PON:	808485(ATP6)

NLE:	16545236(ATP6)

HMH:	116810171 116810507

MCC:	2846629(ATP6)

MCF:	7857742(ATP6)

CSAB:

4097493(ATP6)

CATY:

26408628(ATP6)

PANU:

14444634(ATP6)

TGE:	14216786(ATP6)

RRO:	4171539(ATP6)

RBB:	10549372(ATP6)

TFN:	117075856 117075878 18985077(ATP6)

CJC:	22203905(ATP6)

SBQ:	13228916(ATP6)

CSYR:

7944466(ATP6)

MMUR:

26823979(ATP6)

LCAT:

68929161(ATP6)

PCOQ:

6458123(ATP6)

MMU:	17705(ATP6)

MCAL:

20832247(ATP6)

MPAH:

36165340(ATP6)

RNO:	26197(ATP6)

MCOC:

34683005(ATP6)

MUN:	18129809(ATP6)

CGE:	3979188(ATP6)

MAUA:

8457928(ATP6)

PROB:

30213849(BOP69_gp08)

PLEU:

36275531(ATP6)

NGI:	15088428(ATP6)

HGL:	10201211(ATP6)

CPOC:

808630(ATP6)

CCAN:

31082909(ATP6)

OCU:	808232(ATP6)

OPI:	2717272(ATP6)

GVR:	804974(ATP6)

CFA:	804488(ATP6)

VVP:	4355751(ATP6)

VLG:	23629751(ATP6)

NPO:	8690245(ATP6)

AML:	5179731(ATP6)

UMR:	804865(ATP6)

UAR:	804838(ATP6)

LLV:	71922418(ATP6)

MNP:	20466143(ATP6)

NVS:	14841844(ATP6)

EJU:	805013(ATP6)

ZCA:	4355788(ATP6)

MLX:	4355864(ATP6)

LWW:	4355826(ATP6)

FCA:	807932(ATP6)

PYU:	26129851(ATP6)

PCOO:

11473383(ATP6)

PBG:	26129471(ATP6)

PVIV:

26129623(ATP6)

PPAD:

6262372(ATP6)

PUC:	6262402(ATP6)

AJU:	2654017(ATP6)

HHV:	14842160(ATP6)

BTA:	3283882(ATP6)

BOM:	22161771(ATP6)

BIU:	2885970(ATP6)

BBUB:

56055835(ATP6)

CHX:	1485861(ATP6)

OAS:	808254(ATP6)

BTAX:

8250965(ATP6)

ODA:	11452043(ATP6)

CCAD:

59433124(ATP6)

MBEZ:

7872547(ATP6)

SSC:	808506(ATP6)

CFR:	5326225(ATP6)

CBAI:

5309934(ATP6)

CDK:	5618964(ATP6)

VPC:	809431(ATP6)

BMUS:

807740(ATP6)

LVE:	3802097(ATP6)

OOR:	72909397(ATP6)

DLE:	31413000(ATP6)

PCAD:

809407(ATP6)

PSIU:

116759074

ECB:	807854(ATP6)

EPZ:	19019449(ATP6)

EAI:	808063(ATP6)

MYB:	20964697(ATP6)

MYD:	22203919(ATP6)

MMYO:

26889504(ATP6)

MLF:	27214795(ATP6)

DRO:	17046618(ATP6)

AJM:	808334(ATP6)

PDIC:

74550829(ATP6)

HAI:	13539870(ATP6)

PALE:

17963497(ATP6)

RAY:	3666151(ATP6)

MJV:	24019674(ATP6)

TOD:	38289033(D9W85_mgp08)

LAV:	808788(ATP6)

ETF:	802337(ATP6)

DNM:	808125(ATP6)

MDO:	3074666(ATP6)

GAS:	63652813(ATP6)

SHR:	13826434(ATP6)

AFZ:	65342057(ATP6)

PCW:	4108294(ATP6)

OAA:	808705(ATP6)

GGA:	39116912(ATP6)

PCOC:

10609900(ATP6)

MGP:	5848003(ATP6)

CJO:	804661(ATP6)

LMUT:

31079443(ATP6)

NMEL:

32282767(ATP6)

APLA:

5405819(ATP6)

CATA:

7996702(ATP6)

AFUL:

19909400(ATP6)

TGU:	3950795(ATP6)

SCAN:

37275785(ATP6)

PMOA:

20355701(ATP6)

OTC:	22548463(ATP6)

PRUF:

17674385(ATP6)

FAB:	16027878(ATP6)

PHI:	9480997(ATP6)

PMAJ:

39089561(ATP6)

CCW:	71712386(ATP6)

MMEA:

20357137(ATP6)

FPG:	808596(ATP6)

FCH:	23808602(ATP6)

CLV:	8890000(ATP6)

EGZ:	18985252(ATP6)

NNI:	4108692(ATP6)

PADL:

26737710(ATP6)

AGEN:

7256433(ATP6)

GSTE:

3416065(ATP6)

RTD:	84348659(ATP6)

AROW:

62676609(ATP6)

NPD:	62676468(ATP6)

DNE:	803264(ATP6)

ASN:	806137(ATP6)

AMJ:	808244(ATP6)

CPOO:

4108084(ATP6)

GGN:	4178382(ATP6)

PSS:	2943126(ATP6)

CMY:	808648(ATP6)

CCAY:

11747792(ATP6)

CPIC:

18982999(ATP6)

CABI:

60253679(ATP6)

ACS:	6385984(ATP6)

PVT:	3338346(ATP6)

PBI:	YP_008083604

PMUR:

15822635(ATP6)

APRI:

131187387

VKO:

123021453

PMUA:

7056465(ATP6)

ZVI:	24146079(YC27_gp08)

HCG:

128339205

GJA:	26044406(ATP6)

STOW:

125434148

EMC:	30861793(ATP6)

XLA:	2642083(ATP6)

XTR:	3283497(ATP6)

NPR:	24020216(ATP6)

BGAR:

4356062(ATP6)

MUO:	18490111(ATP6)

GSH:	14469874(ATP6)

DRE:	140519(ATP6)

SRX:	24419175(ATP6)

SANH:

24419949(ATP6)

SGH:	8382167(ATP6)

CCAR:

807763(ATP6)

CGIB:

9222069(ATP6)

PTET:

5787384(ATP6)

LROH:

12079595(ATP6)

OMC:	18668122(ATP6)

PPRM:

26048321(ATP6)

MAMB:

5912294(ATP6)

CIDE:

5857707(ATP6)

MASI:

3131138(ATP6)

TROS:

14048440(ATP6)

TDW:	36952168(ATP6)

MANU:

6879714(ATP6)

IPU:	804877(ATP6)

IFU:	26118464(ATP6)

PHYP:

16216572(ATP6)

SMEO:

10079902(ATP6)

TVC:	10079791(ATP6) 132858796

CHAR:

5228435(ATP6)

TRU:	805215(ATP6)

TFS:	19524536(ATP6)

TNG:	BAE79222(ATPase_6)

LCO:	7095386(ATP6)

NCC:	10752027(ATP6)

PGEO:

67284262(ATP6)

GACU:

117546373

EMAC:

30862043(ATP6)

ELY:	7095521(ATP6)

EFO:	14412332(ATP6)

PLEP:

4363287(ATP6)

SLUC:

23629807(ATP6)

ESP:	40141411(ATP6)

PFLV:

14048222(ATP6)

PPUG:

119199129 7042825(ATP6)

AFB:	13230433(ATP6)

PSWI:

72130124(ATP6)

MSAM:

4100030(ATP6)

SCHU:

10965115(ATP6)

CUD:	8774889(ATP6)

MZE:	26037621(APK84_gp08)

ONL:	8677316(ATP6)

OAU:	8745944(ATP6)

OLA:	805435(ATP6)

OML:	13540112(ATP6)

CSAI:

804091(ATP6)

XMA:	6986205(ATP6)

XCO:	26048169(ATP6)

XHE:	8302511(ATP6)

PRET:

19590710(ATP6)

GAF:	805454(ATP6)

CVG:	26048359(ATP6)

CTUL:

26129129(ATP6)

NFU:	7256401(ATP6)

KMR:	804464(ATP6)

AOCE:

4850671(ATP6)

CSEM:

7996484(ATP6)

POV:	808846(ATP6)

SSEN:

4266839(ATP6)

HHIP:

5405700(ATP6)

HSP:	5405773(ATP6)

SMAU:

8382077(ATP6)

LCF:	3703592(ATP6)

SDU:	11669691(ATP6)

XGL:	7871596(ATP6)

HCQ:	14658015(ATP6)

SSCV:

125966264 125966265

SBIA:

35996564(ATP6)

PTAO:

74193756(ATP6)

BPEC:

YP_004935136.1

MALB:

803931(ATP6)

BSPL:

23631932(ATP6)

SJO:	8703815(ATP6)

SASA:

808313(ATP6)

STRU:

19099180(ATP6)

OTW:	KEF73_p08(ATP6)

OMY:	807976(ATP6)

OGO:	6383207(ATP6)

ONE:	4555714(ATP6)

OKI:	4955080(ATP6)

OKE:	12486864(ATP6)

SALP:

808556(ATP6)

SNH:	35115205(ATP6)

CCLU:

14842636(ATP6)

ELS:	806653(ATP6)

SFM:	3416109(ATP6)

AANG:

3190258(ATP6)

LOC:	807208(ATP6)

PSPA:

805411(ATP6)

ARUT:

17097823(ATP6)

LCM:	808088(ATP6)

CMK:	9385349(ATP6)

RTP:	18267181(ATP6)

CPLA:

7768747(ATP6)

LERI:

11452183(ATP6)

PMRN:

807806(ATP6)

LRJ:	9385083(ATP6)

BFO:	808729(ATP6)

BBEL:

806233(ATP6)

SCLV:

36165649(ATP6)

SPU:	2652722(ATP6)

APLC:

3907686(ATP6)

SKO:	3703604(ATP6)

DME:	Dmel_CG34073(ATP6)

DSE:	2760941(ATP6)

DSI:

ATP6

DYA:

ATP6

CCAT:

110117542 808527(ATP6)

BOD:	2717261(ATP6)

BDR:	4657577(ATP6)

AOQ:	79023081(ATP6)

MDE:	20358591(ATP6)

LSQ:	5469276(ATP6)

ECOE:

35199132(ATP6)

HIS:	33350921(ATP6)

ACOZ:

26121314(ATP6)

AARA:

26121200(ATP6)

AMER:

26121504(ATP6)

AFUN:

76054155(ATP6)

AMOU:

73620849(ATP6)

AALI:

118463451 118464373

AAG:	33307543(ATP6)

AALB:

3260119(ATP6)

CQU:

ATP6

CPII:

10200331(ATP6)

ACER:

9385641(ATP6)

ALAB:

34829140(ATP6)

AFLR:

15822486(ATP6)

BAFF:

126926095

SOC:	9977792(ATP6)

VEM:	27912164(ATP6)

HST:

112589759

LHU:	42265441(ATP6)

PGC:

109863005

VVE:	33194910(ATP6)

TPRE:

111693307

AGIF:

63649823(ATP6)

CCIN:

107274033 7872297(ATP6)

TCA:	803828(ATP6)

SOY:	28481386(ATP6)

ATD:	34926355(ATP6)

CSET:

123313379

AGB:	108912905 4171600(ATP6)

APLN:

28479676(ATP6)

OTU:	111422025 111425816

BMOR:

809269(ATP6)

BMAN:

804656(ATP6)

MSEX:

5848750(ATP6)

DPL:	AGL76302(ATP6)

PMAC:

106711769 13080344(ATP6)

PPOT:

106103433 106110405 20159565(ATP6)

PXU:	106113039 26836897(ATP6)

PRAP:

11030481(ATP6)

PNAP:

60241328(ATP6)

HAW:	9977715(ATP6)

HZE:	71203131(ATP6)

TNL:	113502839 113502842 44149125(ATP6)

SLIU:

17427397(ATP6)

PXY:	20834449(ATP6)

PGW:	73956662(ATP6)

API:	7055878(ATP6)

DNX:	17428412(ATP6)

AGS:	19909103(ATP6)

BTAB:

3077232(ATP6)

DCI:	27914388(ATP6)

CLEC:

27787578(ATP6)

HHAL:

106691405 8457885(ATP6)

NLU:	16216529(ATP6)

HVI:	3332278(ATP6)

MQU:	32891604(ATP6)

FOC:	YP_006576365

TPAL:

38092128(ATP6)

ZNE:	20004827(ATP6)

SGRE:

73491798(ATP6)

DPX:

AAD33234

DMK:	24147188(ATP6)

PVM:	113830130 5333207(ATP6)

PJA:	3416040(ATP6)

PCHN:

5333410(ATP6)

PMOO:

808975(ATP6)

HAME:

10743674(ATP6)

PCLA:

11763613(ATP6)

CQD:	17728515(ATP6)

PTRU:

1482822(ATP6)

ESN:	3355549(ATP6)

MNZ:	10200246(ATP6)

HAZT:

38169105(ATP6)

EAF:	54124840(ATP6)

TCF:	4788310(ATP6)

DSV:	23630420(ATP6)

RSAN:

808366(ATP6)

RMP:	18250938(ATP6)

TUT:	6164208(ATP6)

DPTE:

113797360 7564647(ATP6)

CSCU:

111636256

SDM:

118180532

LPOL:

111086616 803785(ATP6)

CEL:	KEF34_p09(ATP6)

CBR:

ATP6

NAI:	804831(ATP6)

TSP:

ATP6

HRO:	HELRODRAFT_92870

PCAN:

19909182(ATP6)

BGT:	2746316(ATP6)

HRF:	35985150(ATP6)

HRJ:	2846700(ATP6)

CRG:	808827(ATP6)

MYI:	4910441(ATP6)

MMER:

55289780(ATP6)

OBI:	27205961(ATP6)

OSN:	62676627(ATP6)

SMM:

ATP6

ATEN:

41854789(ATP6)

HSY:	130626424 130626456

TAD:	TradoM_p02(ATP6)

ATH:	AT2G07741 DA397_mgp17(atp6) DA397_mgp35(atp6)

CRB:	40490699(atp6)

CSAT:

109133472

BRP:	56141014(atp6)

BNA:	125580701 4237874(atp6)

RSZ:	13630167(atp6)

CPAP:

7441434(atp6)

CIT:	36487038(atp6)

PVY:

116124089

MINC:

123201350 123209026

GRA:	27429608(atp6)

GHI:	24679520(atp6)

GAB:	108487471 33134398(atp6)

EGR:	38466590(atp6)

GMX:	15308536(atp6-1) 15308625(atp6-2)

GSJ:	38334438(atp6) 38334511(atp6)

VAR:	15382779(atp6)

VUN:	114171286 114172402 114188771

CCAJ:

109794084

MTR:	26974351(atp6)

TPRA:

55290673(atp6)

PSAT:

127097794 127107485

VVO:

131637797

LJA:	Ljmtg3v0000190.1(Ljmitog3v0000190.1) Ljmtg3v0000550.1(Ljmitog3v0000550.1)

ADU:	127744285 127744286 127744494 127744647 127745500 127745503

AHF:	112769536 114926184

PCIN:

129294839

PMUM:

103335303

MDM:	114827169 13630235(atp6)

MSYL:

126633984 73938869(atp6)

ZJU:	125421554 27215246(atp6)

CSAV:

27215454(atp6)

CSV:	11123915(atp6)

CMO:	127148204 127149810

RCU:	10221441(atp6) 8287940

MESC:

42317510(atp6)

POP:

18105360

PALZ:

39331246(atp6)

VVI:	132254525 7498515(atp6)

SLY:	101259827 34678325(atp6)

SPEN:

114074752 34926022(atp6)

SDUL:

129892843

CANN:

19989043(atp6)

NTA:	107769558 3205347(atp6)

NSY:	27214832(atp6)

NAU:	35198929(atp6)

INI:	29082063(atp6)

ITR:

116000291

SIND:

105155288

SSPL:

121789895

SMIL:

18126378(atp6)

APAN:

127240797 127240798

HAN:	118483836 118485427 18250997(atp6)

ECAD:

122584389

LSV:	122194923 40498766(atp6)

DCR:	108192759 12598589(atp6)

CSIN:

40867556(atp6)

RVL:

131317609

BVG:	809475(atp6)

SOE:	33876593(atp6)

CQI:	39331961(atp6)

ATRI:

130827046

NNU:	28482659(atp6)

MING:

122067947 122067952 122068889

TSS:	122647065 122647066

OSA:	6450195(atp6)

OGL:

127762842

BDI:

104582115

TDC:	119278428 119278706 119282170 119292773 119307837 119309867 119310787 119313078 119315013 119320327 119333992 119335935 119350401 119350862 119353968

TAES:

123055436 123086424 123136155 123152110 123157488 34688746(atp6-1) 34688752(atp6-2)

TUA:	125508089 125519533 125524096 125524729 125531432 125553662

LPER:

127339782

SBI:	4306003(atp6)

ZMA:	109945965 118472186 118473860 118475694

SITA:

111256056

SVS:

117848565

PVIR:

120639283 120693827 120694098

PDA:	11542582(atp6)

MUS:

ZOF:

DCT:

PEQ:

AOF:

NCOL:

36487317(atp6) 36487372(atp6)

ATR:

110006549

PPP:	3989095(atp6)

VCN:	VOLCADRAFT_110024

CSL:	CospCoM_p11(atp6)

CVR:	OJ20_p24(atp6)

APRO:

ChprMp003(atp6) F751_0126

OTA:	OstapMp30(Atp6)

BPG:	BathyMg00030(atp6)

MIS:	MicpuN_mit18(atp6_1) MicpuN_mit34(atp6_2)

CCP:	ChcroMp20(atp6)

SCE:	Q0085(ATP6)

SPAO:

A560_p02(atp6)

AGO:	AGOS_AMI006W

KLA:	KllafMp03(ATP6)

KMX:	CRD48_mgp04(ATP6)

VPO:	VapofMp08(atp6)

CGR:	CaglfMp09(atp6)

DHA:

ATP6

CAL:	CaalfMp06(ATP6)

YLI:	YalifMp13(atp6)

CLUS:

16792575(atp6)

NCR:

NCU16025

SMP:	SMAC_12686(atp6)

PAN:	PoanfMp10(atp6)

CTHR:

CTHT_m000500

FGR:	GizefMp14(atp6)

PTKZ:

MRV25_mgp04(atp6)

VAL:	VDBG_17001 VDBG_17014

CLUP:

AYB75_mgp01(atp6)

SSL:	SS1G_20039

ANI:	ANID_20010

ANG:	Asnifp07(atp6)

ALUC:

EJ508_mgp11(atp6)

PNO:	SNOGp02(atp6)

TML:	GSTUM_00001101001

SPO:

ScpofMp06

SOM:	PWB26_mgp01(atp6)

ABP:	AGABI1DRAFT17081(AGABI1DRAFT_17081)

MRT:	D9L16_mgp04(atp6)

PGR:	PGTG_21287

PSTR:

EFH26_mgp06(atp6)

DDI:	DidioMp19(atp6)

DFA:	Difao_mp26(atp6)

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Reference

PMID:25037980

Authors

Burrage LC, Tang S, Wang J, Donti TR, Walkiewicz M, Luchak JM, Chen LC, Schmitt ES, Niu Z, Erana R, Hunter JV, Graham BH, Wong LJ, Scaglia F

Title

Mitochondrial myopathy, lactic acidosis, and sideroblastic anemia (MLASA) plus associated with a novel de novo mutation (m.8969G>A) in the mitochondrial encoded ATP6 gene.

Journal

Mol Genet Metab 113:207-12 (2014)
DOI:10.1016/j.ymgme.2014.06.004

Sequence

[hsa:4508]

LinkDB

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