MT-ND5 - mitochondrially encoded NADH dehydrogenase 5 Gene

Also Known as MTND5; ND5

Species: Homo sapiens

Gene Type: protein coding

Gene ID: 4540

Summary

Enables NADH dehydrogenase (ubiquinone) activity. Involved in mitochondrial electron transport, NADH to ubiquinone and mitochondrial respiratory chain complex I assembly. Part of mitochondrial respiratory chain complex I. Implicated in Leber hereditary optic neuropathy; Leigh disease; and MELAS syndrome. [provided by Alliance of Genome Resources, Apr 2022]

MT-ND5 Products (1)

mRNA	Protein	Name
YP_003024036.1 NADH dehydrogenase subunit 5 (mitochondrion) [Homo sapiens]

Gene Ontology

Molecular Function
Biological Process
Cellular Component

Molecular Function GO Annotation	Evidence	References	Source
enables NADH dehydrogenase (ubiquinone) activity	IMP IMP: Inferred from mutant phenotype	15250827	GOA

Biological Process GO Annotation	Evidence	References	Source
involved in mitochondrial electron transport, NADH to ubiquinone	IMP IMP: Inferred from mutant phenotype	15250827	GOA
involved in mitochondrial respiratory chain complex I assembly	IMP IMP: Inferred from mutant phenotype	15250827	GOA

Cellular Component GO Annotation	Evidence	References	Source
located in mitochondrial inner membrane	IDA IDA: Inferred from direct assay	28844695	GOA
part of respiratory chain complex I	IDA IDA: Inferred from direct assay	12611891	GOA

EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

MT-ND5 Protein Structure

Proton_antipo_N

Proton_antipo_M

NADH5_C

0
100
200
300
400
500
603 a.a.

Protein Preferred Names	Protein Names
NADH dehydrogenase subunit 5

MT-ND5 Antibodies

Cat. No.	Product Name	Application	Reactivity
HY-P811123	MT-ND5 Antibody	WB, ICC/IF	Human, Mouse, Rat

Related Diseases

Diseases	Alias
Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, And Stroke-Like Episodes	Melas Syndrome MELAS Mitochondrial Encephalomyopathy Lactic Acidosis And Stroke-Like Episodes Mitochondrial Myopathy, Lactic Acidosis, Stroke-Like Episode Mitochondrial Encephalomyopathy, Lactic Acidosis, And Stroke-Like Episodes Myopathy, Mitochondrial-Encephalopathy-Lactic Acidosis-Stroke Mitochondrial Encephalomyopathy, Lactic Acidosis And Stroke-Like Episodes Mitochondrial Encephalopathy, Lactic Acidosis, And Stroke-Like Episodes Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis And Stroke-Like Episodes Mitochondrial Encephalomyopathy With Lactic Acidosis And Stroke-Like Episodes Syndrome Myopathy, Mitochondrial, Encephalopathy, Lactic Acidosis, And Stroke-Like Episodes
Leber Hereditary Optic Neuropathy, Modifier Of	Leber Optic Atrophy Leber Hereditary Optic Neuropathy LHON Leber'S Hereditary Optic Neuropathy Leber Optic Atrophy, Susceptibility To Leber'S Optic Atrophy LOAM Loas Leber'S Disease Leber'S Optic Neuropathy Optic Atrophy, Hereditary, Leber Lhon, Modifier Of Optic Atrophy, Leber Type Hereditary Optic Neuroretinopathy Leber Hereditary Optic Atrophy Loa Optic Atrophy Leber Type Leber Hereditary Optic Neuropathy, Modifier Leber Hereditary Optic Neuropathy Susceptibility Modifier Of Leber Hereditary Optic Neuropathy Lebers Hereditary Optic Neuropathy Leber Congenital Amaurosis
Leigh Syndrome	Leigh Disease Infantile Subacute Necrotizing Encephalopathy Leigh Syndrome Due To Mitochondrial Complex Iv Deficiency LS Sne Leigh'S Disease Leigh Syndrome Due To Mitochondrial Complex I Deficiency Necrotizing Encephalopathy, Infantile Subacute, Of Leigh Subacute Necrotizing Encephalomyelopathy Necrotizing Encephalopathy Infantile Subacute Of Leigh Leigh Syndrome Due To Mitochondrial Complex Iii Deficiency Infantile Necrotizing Encephalomyelopathy Juvenile Subacute Necrotizing Encephalomyelopathy Leigh'S Necrotizing Encephalopathy Subacute Necrotizing Encephalopathy Juvenile Subacute Necrotizing Encephalopathy Leigh Syndrome Due To Mitochondrial Complex Ii Deficiency Leigh Syndrome Due To Mitochondrial Complex V Deficiency Encephalopathy, Subacute Necrotizing, Infantile Encephalopathy, Subacute Necrotizing, Juvenile Maternally Inherited Leigh Syndrome Subacute Necrotising Encephalomyelopathy Subacute Necrotising Encephalopathy
Myoclonic Epilepsy Associated With Ragged-Red Fibers	Merrf Syndrome MERRF Fukuhara Syndrome Myoclonic Epilepsy Associated With Ragged Red Fibers Myoencephalopathy Ragged-Red Fiber Disease Myoclonic Epilepsy - Ragged Red Fibers Myoclonus Epilepsy And Ragged Red Fibers Myoclonus With Epilepsy And With Ragged Red Fibers Myoclonic Epilepsy With Ragged Red Fibers Myoclonic Epilepsy With Ragged-Red Fibers Fukuhara Disease Myoclonus Epilepsy Associated With Ragged-Red Fibres Myoclonus With Epilepsy With Ragged Red Fibers
Mitochondrial Disease	Mitochondrial Diseases Mitochondrial Disorder
Mitochondrial Myopathy, Infantile, Transient	Mitochondrial Myopathy With Reversible Cytochrome C Oxidase Deficiency MMIT Mitochondrial Myopathy, Infantile, Transient, Due To Respiratory Chain Deficiency Cox Deficiency Myopathy, Infantile, Transient Respiratory Chain Deficiency, Infantile, Transient Benign Cox Deficiency Infantile Reversible Cytochrome C Oxidase Deficiency Myopathy Mitochondrial Myopathy With Reversible Cox Deficiency Mitochondrial Myopathy With Reversible Complex Iv Deficiency Reversible Infantile Cytochrome C Oxidase Deficiency Reversible Infantile Respiratory Chain Deficiency
Mitochondrial Dna-Associated Leigh Syndrome	Mils Maternally Inherited Leigh Syndrome Leigh Disease, Maternally Inherited Subacute Necrotizing Encephalomyelopathy Maternally Inherited Maternally-Inherited Leigh Disease Maternally-Inherited Infantile Subacute Necrotizing Encephalopathy Mtdna-Associated Leigh Syndrome
Leber Plus Disease	Leber Congenital Amaurosis Lca Leber'S Amaurosis Leber'S Disease Amaurosis Congenita Of Leber Amaurosis Congenita Of Leber, Type 1 Lhon Plus Disease Congenital Absence Of The Rods And Cones Congenital Retinal Blindness Crb Congenital Amaurosis Of Retinal Origin Leber'S Congenital Amaurosis Leber Congenital Amaurosis 1 Leber'S Congenital Tapetoretinal Degeneration Leber'S Congenital Tapetoretinal Dysplasia Lca1 Leber Congenital Amaurosis Type 1 Retinal Blindness, Congenital Amaurosis, Leber Congenital Dysgenesis Neuroepithelialis Retinae Hereditary Epithelial Dysplasia Of Retina Hereditary Retinal Aplasia Heredoretinopathia Congenitalis Leber Abiotrophy Leber Congenital Tapetoretinal Degeneration Lebers Congenital Amaurosis Optic Atrophy, Hereditary, Leber
Hereditary Optic Neuropathy
Parkinson Disease 6, Autosomal Recessive Early-Onset	Autosomal Recessive Early-Onset Parkinson Disease 6 Parkinson Disease 6 PARK6 Parkinson Disease 6, Early Onset Parkinson'S Disease 6 Parkinson Disease 6, Early-Onset Autosomal Recessive Early-Onset Parkinson'S Disease 6 Early-Onset Parkinson Disease 6 Autosomal Recessive Early-Onset Parkinson Disease Type 6 Parkinson Disease 6 Early-Onset Parkinson Disease 6 Late-Onset Susceptibility To Parkinson Disease Autosomal Recessive Early-Onset Digenic Pink1/Dj1 Parkinsonism Young Adult Onset Parkinson Disease, Autosomal Recessive Early-Onset, Digenic, Pink1/Dj1 Parkinson Disease, Type 6, Autosomal Recessive, Early-Onset
Mitochondrial Encephalomyopathy	Mitochondrial Encephalomyopathies Encephalomyopathy, Mitochondrial
Kearns-Sayre Syndrome	Ophthalmoplegia Mitochondrial Cytopathy KSS Ophthalmoplegia, Pigmentary Degeneration Of Retina, And Cardiomyopathy Oculocraniosomatic Syndrome Chronic Progressive External Ophthalmoplegia With Myopathy Cpeo With Myopathy Total Ophthalmoplegia Ophthalmoplegia-Plus Syndrome Ophthalmoplegia, Progressive External, With Ragged-Red Fibers Cpeo With Ragged-Red Fibers Oculomotor Paralysis Renal Tubulopathy, Diabetes Mellitus, And Cerebellar Ataxia Due To Duplication O Renal Tubulopathy, Diabetes Mellitus, And Cerebellar Ataxia Due To Duplication Of Mitochondrial Dna Proximal Tubulopathy, Diabetes Mellitus And Cerebellar Ataxia Cpeo With Ragged Red Fibers Ophthalmoplegia Plus Syndrome Ophthalmoplegia, Progressive External, With Ragged Red Fibers Kearns-Sayre Mitochondrial Cytopathy Mitochondrial Myopathies
Progressive Myoclonus Epilepsy 9	Emp9 Pme Type 9 Progressive Myoclonic Epilepsy Due To Lmnb2 Deficiency Progressive Myoclonus Epilepsy Type 9
Neuropathy	Peripheral Neuropathy Peripheral Neuropathies
Progressive Myoclonus Epilepsy 8	Emp8 Pme Type 8 Progressive Myoclonic Epilepsy Due To Cers1 Deficiency Progressive Myoclonus Epilepsy Type 8
Lactic Acidosis	Acidosis, Lactic Acidosis Lactic
Severe Congenital Neutropenia 1
Early Myoclonic Encephalopathy	Myoclonic Epilepsy Myoclonic Seizure Epilepsies, Myoclonic Epileptic Seizures - Myoclonic Epileptic Seizures, Myoclonic Myoclonia Epileptica Myoclonic Seizure Disorder Early Myoclonic Encephalopathy With Suppression-Bursts
Cortical Blindness	Blindness, Cortical
Bile Acid Synthesis Defect, Congenital, 1	CBAS1 Congenital Bile Acid Synthesis Defect 1 3-Beta-Hydroxy-Delta-5-C27-Steroid Oxidoreductase Deficiency Congenital Bile Acid Synthesis Defect Type 1 Basd1 Congenital Bile Acid Synthesis Defect, Type 1 3-Alpha Beta-Hydroxy-Delta-5-C27-Steroid Oxidoreductase, Deficiency Of 3beta-Hsdh Deficiency 3beta-Hydroxy-Delta-5-C27-Steroid Dehydrogenase Deficiency 3beta-Hydroxy-Delta-5-C27-Steroid Oxidoreductase Deficiency Neonatal Progressive Intrahepatic Cholestasis Pfic4 Progressive Familial Intrahepatic Cholestasis Type 4 Bile Acid Synthesis Defect, Congenital, Type 1 Cholestasis, Progressive Familial Intrahepatic 4
Diphyllobothriasis	Diphyllobothrium Infection Fish Tapeworm Bothriocephalosis Infection By Bothriocephalus Fish Tapeworm Infection Dibothriocephalus Anaemia Dibothriocephaliasis Broad Tapeworm Infection Bothriocephaliasis Dibothriocephalus Intestinal Diphyllobothriasis Diphyllobothrium Infestation Dibothriocephalus Infestation Bothriocephalus Infestation Adult Diphyllobothrium Infection Tapeworm Anaemia Infection By Diphyllobothrium Larvae
Wolff-Parkinson-White Syndrome	Wolff-Parkinson-White Pattern Wpw Syndrome Anomalous Atrioventricular Excitation Anomalous A-V Excitation Ventricular Pre-Excitation With Arrhythmia WPWS Ventricular Familial Preexcitation Syndrome Preexcitation Syndrome Ventricular Preexcitation Wpw - [Wolff-Parkinson- White] Syndrome Pre-Excitation Syndrome
Leber Optic Atrophy And Dystonia	LDYT Marsden Syndrome Leber Hereditary Optic Neuropathy With Dystonia Leber Hereditary Optic Neuropathy And Dystonia Familial Dystonia With Visual Failure And Striatal Lucencies Dystonia, Familial, With Visual Failure And Striatal Lucencies Leber Optic Atrophy With Dystonia Dystonia Familial, With Visual Failure And Striatal Lucencies Lhon And Dystonia Leber'S Hereditary Optic Neuropathy With Dystonia
Urocanase Deficiency	Encephalopathy Due To Urocanase Deficiency Urocanate Hydratase Deficiency Urocanic Aciduria UROCD High Urine Urocanic Acid Levels
Parkinsonism	Parkinsonism-Plus Idiopathic Parkinsonism Primary Parkinsonism Paralysis Agitans Syndrome Parkinsonian Syndrome Trembling Paralysis Paralysis Agitans Shaking Palsy Shaking Paralysis
Sparganosis	Infection By Sparganum Larval Diphyllobothriasis Spirometrosis Infection By Spirometra Larvae Larval Dibothriocephaliasis Sparganosis - Larval Diphyllobothriasis Spirometriosis Larval Dibothriocephalus Infestation Larval Diphyllobothrium Infestation Larval Dibothriocephalus Infection Due To Sparganum Larval Fish Tapeworm Infection Spirometra Larvae Infestation Sparganum Infestation Larval Bothriocephalus Infestation
Cystic Echinococcosis	Echinococcus Granulosus Infection Echinococcus Granulosus Infection Of Lung Echinococcus Granulosus Echinococcus Granulosus Infection Of Thyroid Echinococcus Granulosus Infectious Disease Echinococcus Granulosus Infectious Disease Of Liver Echinococcus Granulosus Infectious Disease Of Thyroid Liver Echinococcus Granulosus Lung Echinococcus Granulosus Thyroid Echinococcus Granulosus Unilocular Echinococcosis Unilocular Hydatid Disease Hydatid Disease Hydatidosis Echinococcosis
Coloboma, Ocular, With Or Without Hearing Impairment, Cleft Lip/Palate, And/Or Mental Retardation	Uveal Coloboma-Cleft Lip And Palate-Intellectual Disability COB1 Coloboma-Microphthalmos Syndrome Coloboma-Microphthalmos Syndrome Associated With Sensorineural Hearing Loss, Hematuria, And Cleft Lip/Palate Coloboma, Cleft Lip-Palate And Mental Retardation Syndrome Ocular Coloboma With Or Without Hearing Impairment, Cleft Lip/Palate, And/Or Mental Retardation Uveal Coloboma-Cleft Lip/Palate-Mental Retardation Syndrome Uveal Coloboma-Cleft Lip/Palate-Intellectual Disability Syndrome Coloboma, Ocular, With Or Without Hearing Impairment, Cleft Lip/Palate, And/Or Intellectual Disability Coloboma, Ocular, With/Without Hearing Impairment, Cleft Lip/Palate, And/Or Mental Retardation
Ancylostomiasis	Ankylostomiasis Hookworm Infection Hookworm Infections Ancylostomiasis Due To Ancylostoma Duodenale Ancylostoma Duodenale Infection
Cercarial Dermatitis	Swimmer'S Itch Cutaneous Schistosomiasis Sea Bather'S Eruption Clam-Digger'S Itch Rice-Paddy Itch Sea Bather'S Itch Sawah Itch Schistosomal Cercarial Dermatitis
Dicrocoeliasis	Disease Due To Dicrocoeliidae Lancet Fluke Infection Dicroceliosis Lancet Fluke Disease Lancet Fluke Infestation
Taeniasis	Beef Tapeworm Infection Infection By Taeniarhynchus Saginatus Taenia Saginata Infectious Disease Taenia Saginata Infection Pork Tapeworm Infection Armed Tapeworm Infection Infection By Taenia Solium Intestinal Taenia Solium Infection Pork Tapeworm Taenia Solium Taeniasis Adult Pork Tapeworm Infection Adult Pork Tapeworm Beef Tapeworm Infection By Taenia Saginata Unarmed Tapeworm Infection Taenia Saginata Taeniasis Infection Due To Adult Tapeworm Taenia Saginata Mediocanellata Taenia Infection Infestation By Taenia Saginata Infestation By Taenia Mediocanellata
Coenurosis	Coenuriasis
Myiasis	Infestation By Fly Larvae Infestation By Maggots Maggot Infestation Infestation By Larvae Of Flies Infestation By Dermatobia Infestation By Estrus Ovis Infestation By Oestrus Ovis Oestriasis Oestrosis
Mitochondrial Metabolism Disease	Abnormality Of Mitochondrial Metabolism Mitochondrial Diseases
Cysticercosis	Neurocysticercosis Submacular Cysticercosis Intestinal Taenia Solium Infection Pork Tapeworm Infection Tapeworm Infection: Intestinal Taenia Solum Tapeworm Infection: Pork Tenia Solium Infectious Disease Taeniasis Tenia Solium Infection Cysticerciasis Infection Due To Larval Form Of Taenia Solium
Renal Oncocytoma	Oncocytoma, Renal Oncocytoma Of Kidney Renal Epithelial Oncocytic Tumor Oncocytoma Kidney Oncocytoma Renal Kidney Oncocytoma
Mitochondrial Myopathy	Mitochondrial Myopathies Mitochondrial Cytopathy Myopathies In Mitochondrial Disorders
Mitochondrial Dna Depletion Syndrome 4a	Alpers Syndrome Alpers-Huttenlocher Syndrome Alpers Progressive Infantile Poliodystrophy Alpers Diffuse Degeneration Of Cerebral Gray Matter With Hepatic Cirrhosis Alpers Disease Progressive Sclerosing Poliodystrophy Pndc Diffuse Cerebral Sclerosis Of Schilder MTDPS4A Neuronal Degeneration Of Childhood With Liver Disease, Progressive Alper'S Syndrome Alpers' Disease Or Gray-Matter Degeneration Diffuse Cerebral Degeneration In Infancy Infantile Poliodystrophy Poliodystrophia Cerebri Progressiva Progressive Cerebral Poliodystrophy Alpers' Disease Alpers Progressive Sclerosing Poliodystrophy Progressive Neuronal Degeneration Of Childhood With Liver Disease Ahs Mitochondrial Dna Depletion Syndrome 4a Alpers Type Neuronal Degeneration Of Childhood With Liver Disease Progressive
Optic Nerve Disease	Optic Neuropathy Disorder Of The Second Nerve Optic Nerve Disorder Optic Nerve Abnormality Of The Optic Nerve Optic Nerve Disorders Neuropathy, Optic Disorder Of The Optic Nerve
Cranial Nerve Disease	Cranial Nerve Disorder Disorder Of Cranial Nerve Cranial Nerve Diseases
Mitochondrial Dna Depletion Syndrome	Mtdna Depletion Syndrome
Progressive Myoclonus Epilepsy	Pme Progressive Myoclonic Epilepsy Myoclonic Epilepsies, Progressive Unverricht-Lundborg Syndrome
Parkinson Disease, Late-Onset	Parkinson Disease Parkinson'S Disease PD PARK Parkinson Disease, Susceptibility To Late Onset Parkinson'S Disease Late Onset Parkinson Disease Paralysis Agitans Primary Parkinsonism Idiopathic Parkinson Disease Parkinson'S Parkinson Disease, Late-Onset, Susceptibility To Parkinson Disease, Age Of Onset, Modifier Lewy Body Parkinson Disease Idiopathic Parkinson'S Disease Pd - [Parkinson Disease] Parkinson Disease Nos Parkinson, Nos Primary Parkinson Disease

Diseases	Alias
Waldenstroem'S Macroglobulinemia	Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma

Related Agents

Pre-clinical Phase

Pre-clinical Phase

Bioactive Molecules (3)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-RS21159	mt-Nd5 Mouse Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS08801	MT-ND5 Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS27675	mt-Nd5 Rat Pre-designed siRNA Set A	Pre-designed Set	/	Unkown