KEGG ORTHOLOGY: K03880

ORTHOLOGY: K03880

Entry

K03880                      KO

Symbol

ND3

Name

NADH-ubiquinone oxidoreductase chain 3 [EC:7.1.1.2]

Pathway

map00190

Oxidative phosphorylation

map01100

Metabolic pathways

map04714

Thermogenesis

map04723

Retrograde endocannabinoid signaling

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

M00142

NADH:ubiquinone oxidoreductase, mitochondria

Reaction

R11945

NADH:ubiquinone oxidoreductase

Disease

H00473

Mitochondrial complex I deficiency

H01355

Kearns-Sayre syndrome

H01365

Leber hereditary optic neuropathy and dystonia

Brite

KEGG Orthology (KO) [BR:ko00001]
09100 Metabolism
  09102 Energy metabolism
   00190 Oxidative phosphorylation
    K03880  ND3; NADH-ubiquinone oxidoreductase chain 3
09150 Organismal Systems
  09156 Nervous system
   04723 Retrograde endocannabinoid signaling
    K03880  ND3; NADH-ubiquinone oxidoreductase chain 3
  09159 Environmental adaptation
   04714 Thermogenesis
    K03880  ND3; NADH-ubiquinone oxidoreductase chain 3
09160 Human Diseases
  09161 Cancer: overview
   05208 Chemical carcinogenesis - reactive oxygen species
    K03880  ND3; NADH-ubiquinone oxidoreductase chain 3
  09164 Neurodegenerative disease
   05010 Alzheimer disease
    K03880  ND3; NADH-ubiquinone oxidoreductase chain 3
   05012 Parkinson disease
    K03880  ND3; NADH-ubiquinone oxidoreductase chain 3
   05014 Amyotrophic lateral sclerosis
    K03880  ND3; NADH-ubiquinone oxidoreductase chain 3
   05016 Huntington disease
    K03880  ND3; NADH-ubiquinone oxidoreductase chain 3
   05020 Prion disease
    K03880  ND3; NADH-ubiquinone oxidoreductase chain 3
   05022 Pathways of neurodegeneration - multiple diseases
    K03880  ND3; NADH-ubiquinone oxidoreductase chain 3
  09166 Cardiovascular disease
   05415 Diabetic cardiomyopathy
    K03880  ND3; NADH-ubiquinone oxidoreductase chain 3
09180 Brite Hierarchies
  09182 Protein families: genetic information processing
   03029 Mitochondrial biogenesis
    K03880  ND3; NADH-ubiquinone oxidoreductase chain 3
Enzymes [BR:ko01000]
7. Translocases
  7.1  Catalysing the translocation of protons
   7.1.1  Linked to oxidoreductase reactions
    7.1.1.2  NADH:ubiquinone reductase (H+-translocating)
     K03880  ND3; NADH-ubiquinone oxidoreductase chain 3
Mitochondrial biogenesis [BR:ko03029]
Mitochondrial DNA transcription, translation, and replication factors
  Mitochondrial DNA-encoded proteins
   Mitochondrial respiratory chain complex I
    K03880  ND3; NADH-ubiquinone oxidoreductase chain 3

Other DBs

GO:

0008137

TC:

3.D.1.6

Genes

HSA:

4537(ND3)

PTR:	807870(ND3)

PPS:	807879(ND3)

GGO:	6742686(ND3)

PON:	808479(ND3)

PPYG:

807902(ND3)

NLE:	16545229(ND3)

SSYN:

9072794(ND3)

MCC:	2846622(ND3)

MCF:	7857744(ND3)

CSAB:

4097495(ND3)

CATY:

26408647(ND3)

PANU:

14444636(ND3)

TGE:	14216788(ND3)

RRO:	4171541(ND3)

RBB:	10549374(ND3)

TFN:	18985079(ND3)

CJC:	22203907(ND3)

SBQ:	13228918(ND3)

ANAN:

13230273(ND3)

CSYR:

7944463(ND3)

MMUR:

26823995(ND3)

LCAT:

68929186(ND3)

PCOQ:

6458120(ND3)

MMU:	17718(ND3)

MCAL:

20832249(ND3)

MPAH:

36165337(ND3)

RNO:	26199(ND3)

MCOC:

34683008(ND3)

MUN:	18129811(ND3)

CGE:	3979190(ND3)

MAUA:

8457930(ND3)

PROB:

30213875(BOP69_gp06)

PLEU:

36275556(ND3)

NGI:	15088430(ND3)

HGL:	10201214(ND3)

CPOC:

808639(ND3)

CCAN:

31082912(ND3)

OCU:	808221(ND3)

OPI:	2717278(ND3)

GVR:	804971(ND3)

CFA:	804481(ND3)

VVP:	4355759(ND3)

VLG:	23629753(ND3)

NPO:	8690247(ND3)

AML:	5179736(ND3)

UMR:	804863(ND3)

UAR:	804845(ND3)

LLV:	71922421(ND3)

MNP:	20466145(ND3)

NVS:	14841846(ND3)

EJU:	805018(ND3)

ZCA:	4355796(ND3)

MLX:	4355872(ND3)

LWW:	4355834(ND3)

FCA:	807938(ND3)

PYU:	26129876(ND3)

PCOO:

11473385(ND3)

PBG:	26129496(ND3)

PVIV:

26129648(ND3)

PPAD:

6262373(ND3)

PUC:	6262404(ND3)

AJU:	2654022(ND3)

HHV:	14842162(ND3)

BTA:	3283884(ND3)

BOM:	22161773(ND3)

BIU:	2885978(ND3)

BBUB:

56055858(ND3)

CHX:	1485863(ND3)

OAS:	808255(ND3)

BTAX:

8250967(ND3)

ODA:	11452045(ND3)

CCAD:

59433141(ND3)

MBEZ:

7872549(ND3)

SSC:	808508(ND3)

CFR:	5326226(ND3)

CBAI:

5309944(ND3)

CDK:	5618962(ND3)

VPC:	809423(ND3)

BMUS:

807741(ND3)

LVE:	3802099(ND3)

OOR:	72909422(ND3)

DLE:	31412989(ND3)

PCAD:

809409(ND3)

ECB:	807855(ND3)

EPZ:	19019452(ND3)

EAI:	808060(ND3)

MYB:	20964699(ND3)

MYD:	22203921(ND3)

MMYO:

26889529(ND3)

MLF:	27214821(ND3)

DRO:	17046637(ND3)

AJM:	808335(ND3)

PDIC:

74550855(ND3)

HAI:	13539872(ND3)

PALE:

17963500(ND3)

RAY:	3666159(ND3)

MJV:	24019676(ND3)

TOD:	38289022(D9W85_mgp06)

LAV:	808786(ND3)

ETF:	802332(ND3)

DNM:	808128(ND3)

MDO:	3074658(ND3)

GAS:	63652839(ND3)

SHR:	13826438(ND3)

AFZ:	65342083(ND3)

PCW:	4108304(ND3)

TVP:	803532(ND3)

OAA:	808710(ND3)

GGA:	39116938(ND3)

PCOC:

10609902(ND3)

MGP:	5848008(ND3)

CJO:	804659(ND3)

LMUT:

31079469(ND3)

NMEL:

32282793(ND3)

APLA:

5405822(ND3)

ACYG:

18887305(ND3)

CATA:

7996704(ND3)

AFUL:

19909402(ND3)

TGU:	3950797(ND3)

SCAN:

37275793(ND3)

PMOA:

20355703(ND3)

OTC:	22548465(ND3)

PRUF:

17674381(ND3)

FAB:	16027881(ND3)

PHI:	9480999(ND3)

PMAJ:

39089587(ND3)

CCW:	71712352(ND3)

MMEA:

20357139(ND3)

SATI:

5846213(ND3)

FPG:	808587(ND3)

FCH:	23808604(ND3)

AGEN:

7256436(ND3)

EGZ:	18985254(ND3)

NNI:	4108700(ND3)

PADL:

26737705(ND3)

GSTE:

3416054(ND3)

CLV:	8890003(ND3)

RTD:	84348662(ND3)

AROW:

62676606(ND3)

NPD:	62676471(ND3)

DNE:	803262(ND3)

ASN:	806131(ND3)

AMJ:	808236(ND3)

CPOO:

4108092(ND3)

GGN:	4178390(ND3)

PSS:	2943125(ND3)

CMY:	808647(ND3)

CCAY:

11747798(ND3)

CPIC:

18983001(ND3)

CABI:

60253695(ND3)

ACS:	6385979(ND3)

PVT:	3338374(ND3)

PBI:	YP_008083606

PMUR:

15822637(ND3)

PMUA:

7056467(ND3)

ZVI:	24146104(YC27_gp06)

GJA:	26044431(ND3)

EMC:	30861821(ND3)

XLA:	2642085(ND3)

XTR:	3283499(ND3)

NPR:	24020219(ND3)

BGAR:

4356064(ND3)

MUO:	18490112(ND3)

GSH:	14469876(ND3)

DRE:	140533(ND3)

SRX:	24419171(ND3)

SANH:

24419970(ND3)

SGH:	8382169(ND3)

CCAR:

807771(ND3)

CGIB:

9222071(ND3)

PTET:

5787381(ND3)

LROH:

12079597(ND3)

OMC:	18668124(ND3)

PPRM:

26048346(ND3)

MAMB:

5912299(ND3)

CIDE:

5857711(ND3)

CERY:

20160552(ND3)

MASI:

3131137(ND3)

TROS:

14048442(ND3)

TDW:	36952194(ND3)

MANU:

6879716(ND3)

IPU:	804878(ND3)

IFU:	26118489(ND3)

PHYP:

16216574(ND3)

SMEO:

10079904(ND3)

TVC:	10079793(ND3)

CHAR:

5228439(ND3)

TRU:	805224(ND3)

TFS:	19524538(ND3)

TNG:	BAE79224(ND3)

LCO:	7095388(ND3)

NCC:	10752029(ND3)

PGEO:

67284265(ND3)

EMAC:

30862046(ND3)

ELY:	7095523(ND3)

EFO:	14412334(ND3)

PLEP:

4363289(ND3)

SLUC:

23629809(ND3)

ESP:	40141438(ND3)

PFLV:

14048225(ND3)

PPUG:

7042821(ND3)

AFB:	13230435(ND3)

PSWI:

72130143(ND3)

MSAM:

4100032(ND3)

SCHU:

10965122(ND3)

CUD:	8774891(ND3)

MZE:	26037646(APK84_gp06)

ONL:	8677318(ND3)

OAU:	8745946(ND3)

OLA:	805439(ND3)

OML:	13540114(ND3)

CSAI:

804083(ND3)

XMA:	6986208(ND3)

XCO:	26048194(ND3)

XHE:	8302513(ND3)

PRET:

19590708(ND3)

GAF:	805455(ND3)

CVG:	26048384(ND3)

CTUL:

26129154(ND3)

NFU:	7256397(ND3)

KMR:	804472(ND3)

AOCE:

4850674(ND3)

CSEM:

7996486(ND3)

POV:	808844(ND3)

SSEN:

4266847(ND3)

HHIP:

5405697(ND3)

HSP:	5405797(ND3)

SMAU:

8382079(ND3)

LCF:	3703594(ND3)

SDU:	11669693(ND3)

XGL:	7871599(ND3)

HCQ:	14658017(ND3)

SBIA:

35996590(ND3)

PTAO:

74193759(ND3)

BPEC:

YP_004935138.1

MALB:

803929(ND3)

BSPL:

23631934(ND3)

SJO:	8703818(ND3)

SASA:

808308(ND3)

STRU:

19099182(ND3)

OTW:	KEF73_p06(ND3)

OMY:	807972(ND3)

OGO:	6383208(ND3)

ONE:	4555716(ND3)

OKI:	4955079(ND3)

OKE:	12486866(ND3)

SALP:

808553(ND3)

SNH:	35115223(ND3)

CCLU:

14842638(ND3)

ELS:	806654(ND3)

SFM:	3416098(ND3)

AANG:

3190260(ND3)

LOC:	807199(ND3)

PSPA:

805403(ND3)

ARUT:

17097825(ND3)

LCM:	808090(ND3)

CMK:	9385351(ND3)

RTP:	18267183(ND3)

CPLA:

7768749(ND3)

LERI:

11452185(ND3)

PMRN:

807807(ND3)

LRJ:	9385086(ND3)

BFO:	808731(ND3)

BBEL:

806236(ND3)

SPU:	2652723(ND3)

APLC:

3907681(ND3)

SKO:	3703606(ND3)

DME:	Dmel_CG34076(ND3)

DSE:	2760946(ND3)

DSI:

ND3

DYA:

ND3

CCAT:

808524(ND3)

BOD:	2717256(ND3)

BDR:	4657571(ND3)

AOQ:	79023084(ND3)

MDE:	20358595(ND3)

LSQ:	5469262(ND3)

ECOE:

35199152(ND3)

HIS:	33350932(ND3)

AGA:	85940922(ND3)

ACOZ:

26121324(ND3)

AARA:

26121210(ND3)

AMER:

26121514(ND3)

AFUN:

76054174(ND3)

AMOU:

73620852(ND3)

AAG:	33307561(ND3)

AALB:

3260120(ND3)

CQU:

ND3

CPII:

10200333(ND3) 120418467

ACER:

9385643(ND3)

ALAB:

34829164(ND3)

AFLR:

15822488(ND3)

SOC:	9977794(ND3)

VEM:	27912156(ND3)

LHU:	42265446(ND3)

VVE:	33194929(ND3)

AGIF:

63649826(ND3)

CCIN:

7872295(ND3)

TCA:	803821(ND3)

SOY:	28481405(ND3)

ATD:	34926369(ND3)

AGB:	4171602(ND3)

APLN:

28479679(ND3)

BMOR:

809257(ND3)

BMAN:

804648(ND3)

MSEX:

5848752(ND3)

DPL:	AGL76304(ND3)

PMAC:

13080346(ND3)

PPOT:

20159559(ND3)

PXU:	106113038 26836904(ND3)

PRAP:

11030483(ND3)

PNAP:

60241347(ND3)

HAW:	9977717(ND3)

HZE:	71203134(ND3)

TNL:	44149103(ND3)

SLIU:

17427399(ND3)

ATRA:

26281596(ND3)

GMW:	26376092(ND3)

PXY:	20834451(ND3)

PGW:	73956682(ND3)

API:	7055880(ND3)

DNX:	17428414(ND3)

AGS:	19909105(ND3)

BTAB:

3077224(ND3)

DCI:	27914407(ND3)

CLEC:

27787561(ND3)

HHAL:

8457887(ND3)

NLU:	16216531(ND3)

HVI:	3332279(ND3)

MQU:	32891623(ND3)

FOC:	YP_006576371

TPAL:

38092108(ND3)

ZNE:	20004822(ND3)

SGRE:

73491801(ND3)

DPX:

AAD33236

DMK:	24147209(ND3)

PVM:	5333208(ND3)

PJA:	3416048(ND3)

PCHN:

5333408(ND3)

PMOO:

808983(ND3)

HAME:

10743676(ND3)

PCLA:

11763615(ND3)

CQD:	17728518(ND3)

PTRU:

1482830(ND3)

ESN:	3355541(ND3)

MNZ:	10200248(ND3)

HAZT:

38169111(ND3)

EAF:	54124849(ND3)

TCF:	4788306(ND3)

DSV:	23630422(ND3)

RSAN:

808369(ND3)

RMP:	18250940(ND3)

VDE:	806160(ND3)

TUT:	6164205(ND3)

DPTE:

7564649(ND3)

LPOL:

803784(ND3)

CEL:	KEF34_p02(ND3)

CBR:

ND3

NAI:	804824(ND3)

LOA:

ND3

TSP:

ND3

HRO:	HELRODRAFT_147263 HELRODRAFT_83278

PCAN:

19909189(ND3)

BGT:	2746314(ND3)

HRF:	35985178(ND3)

HRJ:	2846691(ND3)

CRG:	808834(ND3)

CANU:

7865915(ND3)

MYI:	4910449(ND3)

MMER:

55289781(ND3)

RPHI:

29141291(ND3)

OBI:	27205954(ND3)

OSN:	62676632(ND3)

LJP:	79134712(ND3)

LAK:	C2G87_mgp05(ND3)

SMM:

ND3

SHX:	4097439(ND3)

EGL:	41697857(nad3)

ATEN:

41854779(ND3)

ADF:	17674596(ND3)

DGT:	8656269(ND3)

HMG:	6904242(ND3)

RES:	33911767(ND3)

TAD:	TradoM_p20(ND3)

AQU:	4794358(ND3)

ATH:	AT2G07751 DA397_mgp14(nad3)

CRB:	40490674(nad3)

BRP:	56141050(nad3)

BNA:	4237970(nad3)

RSZ:	13630152(nad3)

CPAP:

110811535 7441453(nad3)

CIT:	36487058(nad3)

PVY:

116120443

GRA:	27429577(nad3)

GHI:	24679495(nad3)

GAB:	33134407(nad3)

HSYR:

120174379

EGR:	38466610(nad3)

GMX:	15308563(nad3)

GSJ:	114381770 38334428(nad3)

VAR:	108328560 15382775(nad3)

VUN:

114172421

VUM:

124827006

CCAJ:

109794153

MTR:	26974348(nad3)

TPRA:

55290704(nad3)

CAM:	101509836 105852384

VVO:	131620563 131637802

ADU:	127741637 127743779 127744546 127744549 127744643 127744646

PCIN:

129294883

PPER:

109946550

MDM:	114825879 13630211(nad3)

MSYL:

126630203 73938899(nad3)

ZJU:	125420105 27215256(nad3)

MNT:	39338972(nad3)

CSAV:

115722799

CSV:	11123864(nad3)

CMO:

127151218

BHJ:	120084052 120088816 120089847

CPEP:

111804780

RCU:	10221422(nad3)

HBR:

110668638

MESC:

42317524(nad3)

POP:

127905547

PALZ:

39331229(nad3)

QLO:

115986346

VVI:	7498617(nad3)

SLY:	34678261(nad3)

SPEN:

34925990(nad3)

CANN:

19989078(nad3)

LBB:

132628841

NTA:	3205228(nad3) 3205293(nad3)

NSY:	27214840(nad3) 27214872(nad3)

NAU:	35198920(nad3)

INI:	29082058(nad3)

SMIL:

18126381(nad3)

SHIS:

125209450

APAN:

127243665 127244389 127244554 127264281

HAN:	118480619 18250973(nad3)

ECAD:

122584418 122584442 122585904

LSV:	122194656 122196600 40498723(nad3)

CCAV:

112508223

DCR:	135150740 88614170(nad3)

CSIN:

40867539(nad3)

RVL:

131326193

AEW:	88577567(nad3)

BVG:	809522(nad3)

SOE:	33876573(nad3)

CQI:	39331950(nad3)

ATRI:

130827025

NNU:	28482682(nad3)

MING:

122088858

TSS:	122647085 122647150

OSA:	6450168(nad3)

OGL:

127762845

TDC:	119267845 119281453 119287974 119298826 119301650 119305087 119305296 119320156 119330349 119330818 119331025 119360889

TAES:

123039220 123044167 123044193 123055368 123060962 123096778 123104777 123115836 123120122 123121382 123124396 123125696 123134074 123137272 123148302 123167354 123175587 123180905 34688769(nad3)

TUA:	125523200 125525142 125530880 125533751 125539733 125541803 125549475 125554005

LPER:

127298048 127312611 127349089

SBI:	4306053(nad3)

PVIR:

120693848 120693849 120693870 120694122 120703472 120703539

PHAI:

112880714

PDA:	11542580(nad3)

EGU:

MUS:

DCT:

PEQ:

AOF:

NCOL:

116267892 36487370(nad3)

ATR:

110006942

SMO:	SELMODRAFT_9546

PPP:	3989056(nad3)

VCN:	VOLCADRAFT_82032(nuo3)

CSL:	CospCoM_p13(nad3)

CVR:	OJ20_p21(nad3)

APRO:

ChprMp004(nad3) F751_0125

OTA:	OstapMp16(nad3)

BPG:	BathyMg00155(nad3)

MIS:	MicpuN_mit79(nad3)

CCP:	ChcroMp13(nad3)

DHA:

ND3

LBG:	89432250(nad3)

CAL:	CaalfMp10(NAD3)

CLUS:

16792611(nad3)

YLI:	802603(nd3)

SLB:	AWJ20_5373

NCR:

NCU16007

PAN:	PoanfMp05(nad3)

CTHR:

CTHT_m000280

SSCK:

SPSK_11047

FGR:	GizefMp04(nad3)

CMT:

CCM_09677

PTKZ:

MRV25_mgp14(nad3)

VAL:	VDBG_17005

CLUP:

AYB75_mgp07(nad3)

SSL:	SS1G_20044

ANI:	ANID_20018

ANG:	Asnifp12(nad3)

ALUC:

EJ508_mgp06(nad3)

PNO:	SNOGp12(nad3)

BZE:	COCCADRAFT_46460

BOR:	COCMIDRAFT_43615

ABP:	AGABI1DRAFT48394(AGABI1DRAFT_48394)

MRT:	D9L16_mgp14(nad3)

PSTR:

EFH26_mgp08(nad3)

DDI:	DidioMp13(nad3)

DFA:	Difao_mp22(nad3)

TET:	TepyoMp26(nad3)

RSIN:

B6N60_00461

» show all

Reference

PMID:17152068

Authors

Sarzi E, Brown MD, Lebon S, Chretien D, Munnich A, Rotig A, Procaccio V

Title

A novel recurrent mitochondrial DNA mutation in ND3 gene is associated with isolated complex I deficiency causing Leigh syndrome and dystonia.

Journal

Am J Med Genet A 143A:33-41 (2007)
DOI:10.1002/ajmg.a.31565

Sequence

[hsa:4537]

LinkDB

DBGET integrated database retrieval system