Protein family
Protein sequence
Protein function
Publications
Catalytic activity
EC number
EC number description
1.3.5.1
Oxidoreductases;
Acting on the CH-CH group of donors;
With a quinone or related compound as acceptor;
succinate dehydrogenase
| PDB | Resolution (Å) | PDB name |
|---|---|---|
| 7KCL | 3.14 | Full-length human mitochondrial Hsp90 (TRAP1) in complex with SdhB in the presence of AMP-PNP |
| 7KCM | 3.43 | Full-length human mitochondrial Hsp90 (TRAP1) in complex with SdhB in the presence of AMP-PNP |
| 7KLU | 3.5 | Tetrameric human mitochondrial Hsp90 (TRAP1) in the presence of AMP-PNP |
| 7KLV | 3.1 | Full-length human mitochondrial Hsp90 (TRAP1) SpyCatcher/SpyTag-SdhB heterodimer in the presence of AMP-PNP |
GO ontology
|
GO term | GO description |
|---|---|---|
| Biological Process | GO:0009060 | aerobic respiration |
| Biological Process | GO:0006121 | mitochondrial electron transport, succinate to ubiquinone |
| Biological Process | GO:0042776 | proton motive force-driven mitochondrial ATP synthesis |
| Biological Process | GO:0022904 | respiratory electron transport chain |
| Biological Process | GO:0006105 | succinate metabolic process |
| Biological Process | GO:0006099 | tricarboxylic acid cycle |
| Molecular Function | GO:0051537 | 2 iron, 2 sulfur cluster binding |
| Molecular Function | GO:0051538 | 3 iron, 4 sulfur cluster binding |
| Molecular Function | GO:0051539 | 4 iron, 4 sulfur cluster binding |
| Molecular Function | GO:0009055 | electron transfer activity |
| Molecular Function | GO:0102040 | fumarate reductase (menaquinone) |
| Molecular Function | GO:0046872 | metal ion binding |
| Molecular Function | GO:0008177 | succinate dehydrogenase (ubiquinone) activity |
| Molecular Function | GO:0048039 | ubiquinone binding |
| Cellular Component | GO:0005743 | mitochondrial inner membrane |
| Cellular Component | GO:0031966 | mitochondrial membrane |
| Cellular Component | GO:0005749 | mitochondrial respiratory chain complex II, succinate dehydrogenase complex (ubiquinone) |
| Cellular Component | GO:0005739 | mitochondrion |
| Cellular Component | GO:0005654 | nucleoplasm |
| Cellular Component | GO:0005886 | plasma membrane |
| InterPro
|
InterPro name |
|---|---|
| IPR001041 | 2Fe-2S ferredoxin-type iron-sulfur binding domain |
| IPR004489 | Succinate dehydrogenase/fumarate reductase iron-sulphur protein |
| IPR006058 | 2Fe-2S ferredoxin, iron-sulphur binding site |
| IPR009051 | Alpha-helical ferredoxin |
| IPR012675 | Beta-grasp domain superfamily |
| IPR017896 | 4Fe-4S ferredoxin-type, iron-sulphur binding domain |
| IPR017900 | 4Fe-4S ferredoxin, iron-sulphur binding, conserved site |
| IPR025192 | Succinate dehydogenase/fumarate reductase N-terminal |
| IPR036010 | 2Fe-2S ferredoxin-like superfamily |
| Pfam
|
Pfam name |
|---|---|
| PF13085 | 2Fe-2S iron-sulfur cluster binding domain |
| PF13534 | 4Fe-4S dicluster domain |
| Reactome
|
Reactome Name | Node type
|
Reactome Root | Reactome Root Name |
|---|---|---|---|---|
| R-HSA-611105 | Respiratory electron transport | Internal node | R-HSA-1430728 | Metabolism |
| R-HSA-71403 | Citric acid cycle (TCA cycle) | Leaf | R-HSA-1430728 | Metabolism |
| HPO ID
|
HPO name |
|---|---|
| HP:0001371 | Flexion contracture |
| HP:0001290 | Generalized hypotonia |
| HP:0000360 | Tinnitus |
| HP:0002018 | Nausea |
| HP:0003593 | Infantile onset |
| HP:0002123 | Generalized myoclonic seizure |
| HP:0001482 | Subcutaneous nodule |
| HP:0001626 | Abnormality of the cardiovascular system |
| HP:0012733 | Macule |
| HP:0100006 | Neoplasm of the central nervous system |
| HP:0001342 | Cerebral hemorrhage |
| HP:0000771 | Gynecomastia |
| HP:0012032 | Lipoma |
| HP:0010532 | Paroxysmal vertigo |
| HP:0002861 | Melanoma |
| HP:0000093 | Proteinuria |
| HP:0003574 | Positive regitine blocking test |
| HP:0003510 | Severe short stature |
| HP:0002516 | Increased intracranial pressure |
| HP:0003829 | Typified by incomplete penetrance |
| HP:0003006 | Neuroblastoma |
| HP:0000221 | Furrowed tongue |
| HP:0100031 | Neoplasm of the thyroid gland |
| HP:0003202 | Skeletal muscle atrophy |
| HP:0000717 | Autism |
| HP:0003508 | Proportionate short stature |
| HP:0000007 | Autosomal recessive inheritance |
| HP:0012378 | Fatigue |
| HP:0005162 | Abnormal left ventricular function |
| HP:0008069 | Neoplasm of the skin |
| HP:0000076 | Vesicoureteral reflux |
| HP:0002574 | Episodic abdominal pain |
| HP:0007272 | Progressive psychomotor deterioration |
| HP:0000767 | Pectus excavatum |
| HP:0000256 | Macrocephaly |
| HP:0001249 | Intellectual disability |
| HP:0009711 | Retinal capillary hemangioma |
| HP:0003345 | Elevated urinary norepinephrine |
| HP:0001250 | Seizure |
| HP:0011463 | Childhood onset |
| HP:0031284 | Flushing |
| HP:0008872 | Feeding difficulties in infancy |
| HP:0100242 | Sarcoma |
| HP:0001251 | Ataxia |
| HP:0002666 | Pheochromocytoma |
| HP:0000982 | Palmoplantar keratoderma |
| HP:0003701 | Proximal muscle weakness |
| HP:0000252 | Microcephaly |
| HP:0002664 | Neoplasm |
| HP:0002650 | Scoliosis |
| HP:0000957 | Cafe-au-lait spot |
| HP:0025335 | Delayed ability to stand |
| HP:0000975 | Hyperhidrosis |
| HP:0025269 | Panic attack |
| HP:0004322 | Short stature |
| HP:0002415 | Leukodystrophy |
| HP:0005584 | Renal cell carcinoma |
| HP:0002527 | Falls |
| HP:0200034 | Papule |
| HP:0100780 | Conjunctival hamartoma |
| HP:0003577 | Congenital onset |
| HP:0007663 | Reduced visual acuity |
| HP:0003693 | Distal amyotrophy |
| HP:0002015 | Dysphagia |
| HP:0003487 | Babinski sign |
| HP:0007400 | Irregular hyperpigmentation |
| HP:0001293 | Cranial nerve compression |
| HP:0000544 | External ophthalmoplegia |
| HP:0008936 | Axial hypotonia |
| HP:0000405 | Conductive hearing impairment |
| HP:0007378 | Neoplasm of the gastrointestinal tract |
| HP:0001317 | Abnormal cerebellum morphology |
| HP:0002019 | Constipation |
| HP:0002359 | Frequent falls |
| HP:0003639 | Elevated urinary epinephrine |
| HP:0012114 | Endometrial carcinoma |
| HP:0001176 | Large hands |
| HP:0002474 | Expressive language delay |
| HP:0007350 | Hyperreflexia in upper limbs |
| HP:0000639 | Nystagmus |
| HP:0001337 | Tremor |
| HP:0000130 | Abnormality of the uterus |
| HP:0002421 | Poor head control |
| HP:0002239 | Gastrointestinal hemorrhage |
| HP:0003388 | Easy fatigability |
| HP:0005374 | Cellular immunodeficiency |
| HP:0001605 | Vocal cord paralysis |
| HP:0007565 | Multiple cafe-au-lait spots |
| HP:0003528 | Elevated calcitonin |
| HP:0006801 | Hyperactive deep tendon reflexes |
| HP:0008675 | Enlarged polycystic ovaries |
| HP:0001069 | Episodic hyperhidrosis |
| HP:0000365 | Hearing impairment |
| HP:0012402 | Increased urine alpha-ketoglutarate concentration |
| HP:0003001 | Glomus jugular tumor |
| HP:0012817 | Noncompaction cardiomyopathy |
| HP:0002331 | Recurrent paroxysmal headache |
| HP:0000980 | Pallor |
| HP:0000580 | Pigmentary retinopathy |
| HP:0011343 | Moderate global developmental delay |
| HP:0100751 | Esophageal neoplasm |
| HP:0012740 | Papilloma |
| HP:0010614 | Fibroma |
| HP:0002376 | Developmental regression |
| HP:0001903 | Anemia |
| HP:0001095 | Hypertensive retinopathy |
| HP:0030890 | Hyperintensity of cerebral white matter on MRI |
| HP:0012222 | Arachnoid hemangiomatosis |
| HP:0000953 | Hyperpigmentation of the skin |
| HP:0003542 | Increased serum pyruvate |
| HP:0100543 | Cognitive impairment |
| HP:0008629 | Pulsatile tinnitus |
| HP:0006748 | Adrenal pheochromocytoma |
| HP:0002640 | Hypertension associated with pheochromocytoma |
| HP:0001920 | Renal artery stenosis |
| HP:0000618 | Blindness |
| HP:0000478 | Abnormality of the eye |
| HP:0002017 | Nausea and vomiting |
| HP:0001332 | Dystonia |
| HP:0001635 | Congestive heart failure |
| HP:0004390 | Hamartomatous polyposis |
| HP:0000526 | Aniridia |
| HP:0000158 | Macroglossia |
| HP:0002858 | Meningioma |
| HP:0000096 | Glomerular sclerosis |
| HP:0001712 | Left ventricular hypertrophy |
| HP:0001508 | Failure to thrive |
| HP:0000218 | High palate |
| HP:0000036 | Abnormal penis morphology |
| HP:0011976 | Elevated urinary catecholamines |
| HP:0001028 | Hemangioma |
| HP:0001285 | Spastic tetraparesis |
| HP:0003756 | Skeletal myopathy |
| HP:0006731 | Follicular thyroid carcinoma |
| HP:0100749 | Chest pain |
| HP:0000740 | Episodic paroxysmal anxiety |
| HP:0003745 | Sporadic |
| HP:0100723 | Gastrointestinal stroma tumor |
| HP:0001347 | Hyperreflexia |
| HP:0001270 | Motor delay |
| HP:0000726 | Dementia |
| HP:0100743 | Neoplasm of the rectum |
| HP:0040196 | Mild microcephaly |
| HP:0030074 | Chemodectoma |
| HP:0000790 | Hematuria |
| HP:0000995 | Melanocytic nevus |
| HP:0012062 | Bone cyst |
| HP:0003072 | Hypercalcemia |
| HP:0001252 | Hypotonia |
| HP:0001025 | Urticaria |
| HP:0002864 | Paraganglioma of head and neck |
| HP:0031936 | Delayed ability to walk |
| HP:0002373 | Febrile seizure (within the age range of 3 months to 6 years) |
| HP:0009720 | Adenoma sebaceum |
| HP:0006380 | Knee flexion contracture |
| HP:0011166 | Focal myoclonic seizure |
| HP:0006753 | Neoplasm of the stomach |
| HP:0002808 | Kyphosis |
| HP:0002333 | Motor deterioration |
| HP:0001639 | Hypertrophic cardiomyopathy |
| HP:0000988 | Skin rash |
| HP:0002151 | Increased serum lactate |
| HP:0012707 | Elevated brain lactate level by MRS |
| HP:0007083 | Hyperactive patellar reflex |
| HP:0001263 | Global developmental delay |
| HP:0000518 | Cataract |
| HP:0000006 | Autosomal dominant inheritance |
| HP:0002505 | Loss of ambulation |
| HP:0001511 | Intrauterine growth retardation |
| HP:0000750 | Delayed speech and language development |
| HP:0200063 | Colorectal polyposis |
| HP:0008314 | Decreased activity of mitochondrial complex II |
| HP:0003581 | Adult onset |
| HP:0005214 | Intestinal obstruction |
| HP:0001392 | Abnormality of the liver |
| HP:0001618 | Dysphonia |
| HP:0025336 | Delayed ability to sit |
| HP:0005595 | Generalized hyperkeratosis |
| HP:0003324 | Generalized muscle weakness |
| HP:0003002 | Breast carcinoma |
| HP:0006737 | Extraadrenal pheochromocytoma |
| HP:0002668 | Paraganglioma |
| HP:0001156 | Brachydactyly |
| HP:0002313 | Spastic paraparesis |
| HP:0001649 | Tachycardia |
| HP:0001053 | Hypopigmented skin patches |
| HP:0000820 | Abnormality of the thyroid gland |
| HP:0001257 | Spasticity |
| HP:0100579 | Mucosal telangiectasiae |
| HP:0000875 | Episodic hypertension |
| HP:0100273 | Neoplasm of the colon |
| HP:0002027 | Abdominal pain |
| HP:0001067 | Neurofibroma |
| HP:0000853 | Goiter |
| HP:0001824 | Weight loss |
| HP:0100833 | Neoplasm of the small intestine |
| HP:0001048 | Cavernous hemangioma |
| HP:0011979 | Elevated urinary dopamine |
| HP:0000737 | Irritability |
| HP:0000545 | Myopia |
| HP:0011703 | Sinus tachycardia |
| HP:0001962 | Palpitations |
| HP:0006824 | Cranial nerve paralysis |
| HP:0006895 | Lower limb hypertonia |
| HP:0005150 | Abnormal atrioventricular conduction |
| HP:0000077 | Abnormality of the kidney |
| HP:0000519 | Developmental cataract |
| Tissue
|
Cell type
|
Expression level | Reliability |
|---|---|---|---|
| adipose tissue | adipocytes | Low | Enhanced |
| adrenal gland | glandular cells | High | Enhanced |
| appendix | glandular cells | High | Enhanced |
| appendix | lymphoid tissue | Medium | Enhanced |
| bone marrow | hematopoietic cells | Medium | Enhanced |
| breast | adipocytes | Medium | Enhanced |
| breast | glandular cells | High | Enhanced |
| breast | myoepithelial cells | High | Enhanced |
| bronchus | respiratory epithelial cells | High | Enhanced |
| caudate | glial cells | Medium | Enhanced |
| caudate | neuronal cells | High | Enhanced |
| cerebellum | Purkinje cells | Medium | Enhanced |
| cerebellum | cells in granular layer | High | Enhanced |
| cerebellum | cells in molecular layer | Medium | Enhanced |
| cerebral cortex | endothelial cells | Medium | Enhanced |
| cerebral cortex | glial cells | High | Enhanced |
| cerebral cortex | neuronal cells | High | Enhanced |
| cerebral cortex | neuropil | Medium | Enhanced |
| cervix | glandular cells | Medium | Enhanced |
| cervix | squamous epithelial cells | Medium | Enhanced |
| colon | endothelial cells | Medium | Enhanced |
| colon | glandular cells | High | Enhanced |
| colon | peripheral nerve/ganglion | Low | Enhanced |
| duodenum | glandular cells | High | Enhanced |
| endometrium 1 | cells in endometrial stroma | Medium | Enhanced |
| endometrium 1 | glandular cells | High | Enhanced |
| endometrium 2 | cells in endometrial stroma | Medium | Enhanced |
| endometrium 2 | glandular cells | Medium | Enhanced |
| epididymis | glandular cells | Medium | Enhanced |
| esophagus | squamous epithelial cells | High | Enhanced |
| fallopian tube | glandular cells | Medium | Enhanced |
| gallbladder | glandular cells | High | Enhanced |
| heart muscle | cardiomyocytes | High | Enhanced |
| hippocampus | glial cells | High | Enhanced |
| hippocampus | neuronal cells | Medium | Enhanced |
| kidney | bowman's capsule | High | Enhanced |
| kidney | cells in glomeruli | Medium | Enhanced |
| kidney | collecting ducts | High | Enhanced |
| kidney | distal tubules | High | Enhanced |
| kidney | proximal tubules (cell body) | High | Enhanced |
| liver | cholangiocytes | Medium | Enhanced |
| liver | hepatocytes | High | Enhanced |
| lung | alveolar cells type II | Medium | Enhanced |
| lung | macrophages | High | Enhanced |
| lymph node | germinal center cells | Medium | Enhanced |
| lymph node | non-germinal center cells | High | Enhanced |
| nasopharynx | respiratory epithelial cells | High | Enhanced |
| oral mucosa | squamous epithelial cells | Medium | Enhanced |
| ovary | follicle cells | Medium | Enhanced |
| ovary | ovarian stroma cells | Medium | Enhanced |
| pancreas | exocrine glandular cells | High | Enhanced |
| pancreas | pancreatic endocrine cells | Medium | Enhanced |
| parathyroid gland | glandular cells | High | Enhanced |
| placenta | decidual cells | Medium | Enhanced |
| placenta | trophoblastic cells | Medium | Enhanced |
| prostate | glandular cells | Medium | Enhanced |
| rectum | glandular cells | High | Enhanced |
| salivary gland | glandular cells | Medium | Enhanced |
| seminal vesicle | glandular cells | Medium | Enhanced |
| skeletal muscle | myocytes | Medium | Enhanced |
| skin 1 | cells in basal layer | Medium | Enhanced |
| skin 1 | cells in spinous layer | Medium | Enhanced |
| skin 1 | endothelial cells | Medium | Enhanced |
| skin 1 | fibrohistiocytic cells | Medium | Enhanced |
| skin 1 | hair follicles | Medium | Enhanced |
| skin 1 | langerhans cells | Medium | Enhanced |
| skin 1 | lymphocytes | High | Enhanced |
| skin 1 | melanocytes | Medium | Enhanced |
| skin 1 | sebaceous glands | Medium | Enhanced |
| skin 2 | cells in basal layer | Medium | Enhanced |
| skin 2 | cells in spinous layer | Medium | Enhanced |
| skin 2 | endothelial cells | Low | Enhanced |
| skin 2 | fibrohistiocytic cells | Medium | Enhanced |
| skin 2 | langerhans cells | Medium | Enhanced |
| skin 2 | lymphocytes | High | Enhanced |
| skin 2 | melanocytes | Medium | Enhanced |
| small intestine | glandular cells | High | Enhanced |
| smooth muscle | smooth muscle cells | Medium | Enhanced |
| soft tissue 1 | chondrocytes | Medium | Enhanced |
| soft tissue 1 | fibroblasts | Low | Enhanced |
| soft tissue 1 | peripheral nerve | Low | Enhanced |
| soft tissue 2 | fibroblasts | Low | Enhanced |
| soft tissue 2 | peripheral nerve | Low | Enhanced |
| spleen | cells in red pulp | Medium | Enhanced |
| spleen | cells in white pulp | High | Enhanced |
| stomach 1 | glandular cells | High | Enhanced |
| stomach 2 | glandular cells | Medium | Enhanced |
| testis | Leydig cells | High | Enhanced |
| testis | pachytene spermatocytes | High | Enhanced |
| testis | sertoli cells | High | Enhanced |
| testis | spermatogonia cells | High | Enhanced |
| thyroid gland | glandular cells | Medium | Enhanced |
| tonsil | germinal center cells | High | Enhanced |
| tonsil | non-germinal center cells | High | Enhanced |
| tonsil | squamous epithelial cells | Medium | Enhanced |
| urinary bladder | urothelial cells | High | Enhanced |
| vagina | squamous epithelial cells | Medium | Enhanced |
| Disease name | MONDO ID
|
ICD10
|
ICD10 chapter
|
OMIM
|
Orphanet
|
|---|---|---|---|---|---|
| paragangliomas 4 | MONDO:0007273 | C64 | chapter2, Neoplasms | OMIM:115310 | |
| paragangliomas 4 | MONDO:0007273 | C68 | chapter2, Neoplasms | OMIM:115310 | |
| paragangliomas 4 | MONDO:0007273 | C72 | chapter2, Neoplasms | OMIM:115310 | |
| paragangliomas 4 | MONDO:0007273 | C74 | chapter2, Neoplasms | OMIM:115310 | |
| paragangliomas 4 | MONDO:0007273 | C75 | chapter2, Neoplasms | OMIM:115310 | |
| paragangliomas 4 | MONDO:0007273 | D35 | chapter2, Neoplasms | OMIM:115310 | |
| pheochromocytoma | MONDO:0008233 | C74 | chapter2, Neoplasms | OMIM:171300 | |
| pheochromocytoma | MONDO:0008233 | C75 | chapter2, Neoplasms | OMIM:171300 | |
| pheochromocytoma | MONDO:0008233 | D35 | chapter2, Neoplasms | OMIM:171300 | |
| gastrointestinal stromal tumor | MONDO:0011719 | C26 | chapter2, Neoplasms | OMIM:606764 | Orphanet:44890 |
| carney-stratakis syndrome | MONDO:0011740 | D44 | chapter2, Neoplasms | OMIM:606864 | Orphanet:97286 |
| cowden disease | MONDO:0016063 | Q85 | chapter17, Congenital malformations, deformations and chromosomal abnormalities | OMIMPS:158350 | Orphanet:201 |
| kidney neoplasm | MONDO:0021163 | C64 | chapter2, Neoplasms | ||
| kidney neoplasm | MONDO:0021163 | D30 | chapter2, Neoplasms | ||
| mitochondrial complex 2 deficiency, nuclear type 4 | MONDO:0030974 | G71 | chapter6, Diseases of the nervous system | OMIM:619224 | |
| mitochondrial complex ii deficiency, nuclear type 1 | MONDO:0100294 | G71 | chapter6, Diseases of the nervous system | OMIM:252011 | Orphanet:3208 |