MCID: PRT058
MIFTS: 59

Pure Autonomic Failure

Categories: Rare diseases, Cardiovascular diseases, Neuronal diseases

Aliases & Classifications for Pure Autonomic Failure

MalaCards integrated aliases for Pure Autonomic Failure:

Name: Pure Autonomic Failure 53 59 55 73
Idiopathic Orthostatic Hypotension 53 59 73
Bradbury-Eggleston Syndrome 53 54 59
Orthostatic Hypotension 53 54 29
Bradbury Eggleston Syndrome 53
Pure Idiopatic Dysautonomia 59
Hypotension Orthostatic 55
Pure Dysautonomia 59
Paf 59

Characteristics:

Orphanet epidemiological data:

59
pure autonomic failure
Inheritance: Not applicable; Prevalence: 1-9/1000000 (Europe); Age of onset: Adult; Age of death: elderly;

Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

Orphanet 59 ORPHA441
MESH via Orphanet 45 D054970 C544351
UMLS via Orphanet 74 C0393911 C2931939
ICD10 via Orphanet 34 G90.3
ICD10 33 I95.1

Summaries for Pure Autonomic Failure

NINDS : 54 Orthostatic hypotension is a sudden fall in blood pressure that occurs when a person assumes a standing position. It is due to a lesion of the baroreflex loop, which senses a change in blood pressure and adjusts heart rate and activates sympathetic nerve system fibers to cause the blood vessels to narrow and correct blood pressure. It may also be caused by hypovolemia (a decreased amount of blood in the body), resulting from the excessive use of diuretics, vasodilators, or other types of drugs, dehydration, or prolonged bed rest. The disorder may be associated with Addison's disease, diabetes, and certain neurological disorders including Multiple System Atrophy with Orthostatic Hypotension (formerly known as Shy-Drager syndrome), autonomic system neuropathies, and other dysautonomias. Symptoms, which generally occur after sudden standing, include dizziness, lightheadedness, blurred vision, and syncope (temporary loss of consciousness).

MalaCards based summary : Pure Autonomic Failure, also known as idiopathic orthostatic hypotension, is related to multiple system atrophy 1 and autonomic dysfunction. An important gene associated with Pure Autonomic Failure is DBH (Dopamine Beta-Hydroxylase), and among its related pathways/superpathways are Activated PKN1 stimulates transcription of AR (androgen receptor) regulated genes KLK2 and KLK3 and Circadian entrainment. The drugs Midodrine and Droxidopa have been mentioned in the context of this disorder. Affiliated tissues include heart, brain and spinal cord, and related phenotypes are anhidrosis and orthostatic hypotension

NIH Rare Diseases : 53 Pure autonomic failure is characterized by generalized autonomic failure without central nervous system (brain or spinal cord) involvement. The autonomic nervous system is the part of our bodies that controls involuntary actions, such as the widening or narrowing of our blood vessels. Failure of this system can cause a variety of symptoms. The most common symptom of pure autonomic failure is orthostatic hypotension. Other symptoms may include decreased sweating, heat intolerance, inability to empty the bladder, erectile dysfunction, incontinence or constipation, and pupil changes. The cause of this condition is usually unknown.

Wikipedia : 76 Pure autonomic failure (PAF) is a form of dysautonomia that first occurs in middle age or later in life;... more...

Related Diseases for Pure Autonomic Failure

Diseases related to Pure Autonomic Failure via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 91)
# Related Disease Score Top Affiliating Genes
1 multiple system atrophy 1 33.2 DBH SNCA TH
2 autonomic dysfunction 33.2 DBH SNCA
3 rem sleep behavior disorder 30.4 ACHE SNCA
4 postural hypotension 29.8 DBH DDC NPPA REN
5 synucleinopathy 29.8 DDC MAOB SNCA TH
6 parkinson disease, late-onset 27.5 ACHE DBH DDC MAOA MAOB SNCA
7 primary orthostatic hypotension 11.5
8 dysautonomia 11.4
9 platelet-activating factor acetylhydrolase deficiency 11.1
10 neuropathy, hereditary sensory and autonomic, type iii 11.0
11 hereditary dystonia 10.8 DBH TH
12 hyporeninemic hypoaldosteronism 10.8 ACE REN
13 renal artery obstruction 10.8 ACE REN
14 supratentorial primitive neuroectodermal tumor 10.7 DDC TH
15 orthostatic intolerance 10.7
16 hypertensive retinopathy 10.7 ACE REN
17 orthostatic proteinuria 10.7 ACE REN
18 atrial septal defect 4 10.6 ACE REN
19 diabetic autonomic neuropathy 10.6 DBH EPO
20 amyloidosis, hereditary, transthyretin-related 10.5 DBH TTR
21 malignant essential hypertension 10.5 ACE REN
22 movement disease 10.5 DDC SNCA TH
23 anuria 10.5 ACE REN
24 hypoganglionosis 10.5 ACHE TH
25 syndrome of inappropriate antidiuretic hormone 10.5 ACE NPPA
26 hypertensive encephalopathy 10.4 ACE EPO REN
27 autoimmune polyendocrine syndrome type 1 10.4 DDC TH
28 hypoaldosteronism 10.4 ACE NPPA REN
29 hypertension, diastolic 10.4 ACE NPPA REN
30 malignant hypertension 10.4 ACE NPPA REN
31 hepatorenal syndrome 10.4 ACE NPPA REN
32 renovascular hypertension 10.4 ACE NPPA REN
33 urinary system disease 10.4 ACE EPO REN
34 aortic valve disease 2 10.4 ACE NPPA REN
35 postencephalitic parkinson disease 10.3 MAOB SNCA
36 acquired metabolic disease 10.3 ACE REN TTR
37 postural orthostatic tachycardia syndrome 10.3
38 early-onset schizophrenia 10.3 DBH MAOA TH
39 oculogyric crisis 10.3 DDC MAOB
40 gastroesophageal reflux 10.3
41 supranuclear palsy, progressive, 1 10.2 ACHE SNCA TH
42 autonomic nervous system disease 10.2 DBH TTR
43 endotheliitis 10.2
44 renal hypertension 10.2 ACE DBH EPO REN
45 vasculogenic impotence 10.2 ACE NPPA PDE5A
46 pheochromocytoma 10.1 DBH DDC NPPA TH
47 cerebrovascular disease 10.1 ACE ACHE REN
48 idiopathic edema 10.1 ACE NPPA
49 renal artery disease 10.0 ACE EPO NPPA REN
50 intracranial hypotension 10.0

Graphical network of the top 20 diseases related to Pure Autonomic Failure:



Diseases related to Pure Autonomic Failure

Symptoms & Phenotypes for Pure Autonomic Failure

Human phenotypes related to Pure Autonomic Failure:

59 32 (show all 10)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 anhidrosis 59 32 hallmark (90%) Very frequent (99-80%) HP:0000970
2 orthostatic hypotension 59 32 hallmark (90%) Very frequent (99-80%) HP:0001278
3 dysautonomia 59 32 hallmark (90%) Very frequent (99-80%) HP:0002459
4 abnormality of circulating catecholamine level 59 32 hallmark (90%) Very frequent (99-80%) HP:0012099
5 urinary incontinence 59 32 frequent (33%) Frequent (79-30%) HP:0000020
6 syncope 59 32 frequent (33%) Frequent (79-30%) HP:0001279
7 constipation 59 32 frequent (33%) Frequent (79-30%) HP:0002019
8 dysuria 59 32 frequent (33%) Frequent (79-30%) HP:0100518
9 impotence 59 32 occasional (7.5%) Occasional (29-5%) HP:0000802
10 constitutional symptom 59 Very frequent (99-80%)

MGI Mouse Phenotypes related to Pure Autonomic Failure:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 10.13 TH TTR EPO SNCA ACE MAOA
2 behavior/neurological MP:0005386 10.1 TH SNCA ACE MAOA MAOB ACHE
3 cardiovascular system MP:0005385 10.07 TH EPO SNCA ACE MAOA NEDD4L
4 mortality/aging MP:0010768 9.96 SNCA TH REN EPO GNB3 ACE
5 nervous system MP:0003631 9.85 TH SNCA GNB3 MAOA MAOB ACHE
6 renal/urinary system MP:0005367 9.5 SNCA ACE MAOB NEDD4L NPPA DDC
7 vision/eye MP:0005391 9.17 TH TTR GNB3 ACHE NEDD4L DBH

Drugs & Therapeutics for Pure Autonomic Failure

Drugs for Pure Autonomic Failure (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 213)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Midodrine Approved Phase 4,Phase 1 133163-28-7, 42794-76-3 4195
2
Droxidopa Approved, Investigational Phase 4,Phase 3,Phase 2,Phase 1 23651-95-8 443940
3 Neurotransmitter Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable,Early Phase 1
4 Adrenergic Agents Phase 4,Phase 1,Phase 2,Not Applicable,Early Phase 1
5 Adrenergic Agonists Phase 4,Phase 1,Phase 2,Not Applicable
6 Adrenergic alpha-Agonists Phase 4,Phase 1,Phase 2,Not Applicable
7 Vasoconstrictor Agents Phase 4,Phase 1,Phase 2,Early Phase 1,Not Applicable
8 Peripheral Nervous System Agents Phase 4,Phase 3,Phase 1,Phase 2,Not Applicable,Early Phase 1
9 Antiparkinson Agents Phase 4,Phase 3,Phase 2,Phase 1,Not Applicable
10 Autonomic Agents Phase 4,Phase 3,Phase 1,Phase 2,Not Applicable,Early Phase 1
11
Dopamine Approved Phase 3,Phase 2,Phase 1 51-61-6, 62-31-7 681
12
Mannitol Approved, Investigational Phase 3 69-65-8 453 6251
13 Rho(D) Immune Globulin Phase 2, Phase 3
14 gamma-Globulins Phase 2, Phase 3
15 Dopamine Agents Phase 3,Phase 2,Phase 1
16 Antibodies Phase 2, Phase 3
17 Immunoglobulins Phase 2, Phase 3
18 Immunoglobulins, Intravenous Phase 2, Phase 3
19 Protective Agents Phase 3,Phase 1,Not Applicable
20
Fludrocortisone Approved, Investigational Phase 2,Not Applicable 127-31-1 31378
21
Norepinephrine Approved Phase 1, Phase 2,Phase 2 51-41-2 439260
22
Carbidopa Approved Phase 2,Phase 1 28860-95-9 34359 38101
23
Morphine Approved, Investigational Phase 2 57-27-2 5288826
24
Naloxone Approved, Vet_approved Phase 2 465-65-6 5284596
25
Epinephrine Approved, Vet_approved Phase 2,Not Applicable 51-43-4 5816
26
Racepinephrine Approved Phase 2,Not Applicable 329-65-7 838
27 Pyridostigmine Bromide Phase 2,Phase 1 101-26-8
28 Cholinergic Agents Phase 2,Phase 1,Not Applicable
29 Cholinesterase Inhibitors Phase 2,Phase 1
30 Anticonvulsants Phase 2,Phase 1
31 Anti-Inflammatory Agents Phase 2,Phase 1,Not Applicable
32 Bromides Phase 2,Phase 1
33 insulin Phase 2,Phase 1,Not Applicable,Early Phase 1
34 Analgesics Phase 2,Phase 1
35 Respiratory System Agents Phase 2,Phase 1,Not Applicable,Early Phase 1
36 Central Nervous System Depressants Phase 2,Phase 1,Not Applicable
37 Adrenergic beta-Agonists Phase 2,Phase 1,Not Applicable
38 Mydriatics Phase 2,Phase 1,Not Applicable
39 Neurotransmitter Uptake Inhibitors Phase 2,Phase 1,Not Applicable,Early Phase 1
40 Anti-Asthmatic Agents Phase 2,Phase 1,Not Applicable,Early Phase 1
41 Insulin, Globin Zinc Phase 2,Phase 1,Not Applicable,Early Phase 1
42 Aromatic Amino Acid Decarboxylase Inhibitors Phase 2,Phase 1
43 Atomoxetine Hydrochloride Phase 2,Phase 1
44 Bronchodilator Agents Phase 2,Phase 1,Not Applicable,Early Phase 1
45 Narcotic Antagonists Phase 2
46 Narcotics Phase 2
47 Analgesics, Opioid Phase 2
48 Epinephryl borate Phase 2,Not Applicable
49
Acetazolamide Approved, Vet_approved Phase 1 59-66-5 1986
50
Rivastigmine Approved, Investigational Phase 1 123441-03-2 77991

Interventional clinical trials:

(show top 50) (show all 71)
# Name Status NCT ID Phase Drugs
1 A Study for Patients With Neurogenic Orthostatic Hypotension Completed NCT00046475 Phase 4 Midodrine Hydrochloride
2 Sustained Effect of Droxidopa in Symptomatic Neurogenic Orthostatic Hypotension Recruiting NCT02586623 Phase 4 Droxidopa capsules;Placebo capsules
3 A Phase IV Study in Subjects With Neurogenic Orthostatic Hypotension Terminated NCT00046163 Phase 4 midodrine hydrochloride (ProAmatine®)
4 A Clinical Study for Patients With Neurogenic Orthostatic Hypotension (NOH) Using Droxidopa Completed NCT00782340 Phase 3 Placebo;Droxidopa
5 Clinical Study of Droxidopa in Patients With Neurogenic Orthostatic Hypotension (NOH) Completed NCT00633880 Phase 3 Placebo;Droxidopa
6 Clinical Study of Droxidopa in Patients With Neurogenic Orthostatic Hypotension (NOH) (Droxi-304) Completed NCT01132326 Phase 3 Droxidopa
7 Open-Label Clinical Study of Droxidopa in Patients With Neurogenic Orthostatic Hypotension (NOH) Completed NCT00738062 Phase 3 Droxidopa;Placebo
8 Evaluating the Effectiveness of Intravenous Immunoglobulin Therapy in Autoimmune Autonomic Ganglionopathy Completed NCT01522235 Phase 2, Phase 3 Double blinded IVIg
9 A Clinical Study of Patients With Symptomatic NOH to Assess Sustained Effects of Droxidopa Therapy Terminated NCT01927055 Phase 3 Droxidopa;Placebo
10 Orthostatic Dysregulation and Associated Gastrointestinal Dysfunction in Parkinson's Disease -Treatment Unknown status NCT01993680 Phase 2 Pyridostigmine bromide;fludrocortisone
11 Fipamezole in Neurogenic Orthostatic Hypotension Unknown status NCT00758849 Phase 2 Placebo;Fipamezole
12 Acetylcholinesterase Inhibition and Orthostatic Hypotension in SCI Completed NCT02307526 Phase 2 Pyridostigmine Bromide
13 Intravenous Norepinephrine for Orthostatic Hypotension Completed NCT01285908 Phase 1, Phase 2 Intravenous Norepinephrine Infusion
14 Study of Droxidopa Treatment in Adults With Attention Deficit Hyperactivity Disorder With Co-administration of Carbidopa Completed NCT00983814 Phase 2 Droxidopa+Carbidopa;Placebo
15 Norepinephrine Transporter Blockade, Autonomic Failure Recruiting NCT02796209 Phase 2 Atomexetine;Placebo
16 Mechanisms Responsible for Hypoglycemia Associated Autonomic Failure Recruiting NCT00678145 Phase 2 naloxone;Morphine sulfate;Epinephrine
17 Norepinephrine Transporter Blockade, Autonomic Failure (NETAF) Recruiting NCT02784535 Phase 2 Atomoxetine;Placebo
18 Naloxone, Hypoglycemia and Exercise Recruiting NCT03149770 Phase 2 Naloxone;Placebo
19 TD-9855 Phase 2 in Neurogenic Orthostatic Hypotension (nOH) Active, not recruiting NCT02705755 Phase 2 TD-9855;Placebo
20 A Study of Norepinephrine in Patients With Congenital Insensitivity to Pain and Anhidrosis Withdrawn NCT02624310 Phase 2 Droxidopa (L-DOPS);Placebo
21 Assessment of Hand-grip in the Prevention of Postural Orthostatic Hypotension Unknown status NCT01521221 Phase 1
22 Treatment of Orthostatic Hypotension in Autonomic Failure Completed NCT00223691 Phase 1 Atomoxetine;Acarbose;Pyridostigmine Bromide;Yohimbine;Midodrine HCl;placebo;Modafinil;Octreotide;Diphenhydramine Hydrochloride;Ranitidine HCL;Tranylcypromine;Ergotamine/ Caffeine;Celecoxib;Pseudoephedrine;Methylphenidate;Indomethacin;Ibuprofen;Oxymetazoline 0.05% nasal solution;Acetazolamide;Rivastigmine tartrate;Carbidopa/levodopa
23 Treatment of Supine Hypertension in Autonomic Failure Completed NCT00223717 Phase 1 Clonidine;Nitroglycerin transdermal;Dipyridamole/ Aspirin (Aggrenox);Desmopressin (DDAVP);Sildenafil;Nifedipine;Hydralazine;Hydrochlorothiazide;Placebo;Bosentan;Diltiazem;Eplerenone;guanfacine;captopril;carbidopa;losartan;metoprolol tartrate;nebivolol hydrochloride;prazosin hydrochloride;tamsulosin hydrochloride;aliskiren
24 The Autonomic Nervous System and Obesity Completed NCT00179023 Phase 1 Trimethaphan;Trimethaphan;Pseudoephedrine
25 Hemodynamic Mechanisms of Abdominal Compression in the Treatment of Orthostatic Hypotension in Autonomic Failure Recruiting NCT02429557 Phase 1 Placebo pill;midodrine
26 Effects of Midodrine and Droxidopa on Splanchnic Capacitance in Autonomic Failure Recruiting NCT02897063 Phase 1 Droxidopa;Midodrine;Placebo
27 The Role of Endothelin in the Supine Hypertension of Autonomic Failure Enrolling by invitation NCT01119417 Phase 1 BQ123;BQ123;Bq123;BQ123;Saline
28 Liver Glycogen and Hypoglycemia in Humans Not yet recruiting NCT03241706 Phase 1 Fructose;Saline;Somatostatin;Insulin;Glucagon;Dextrose solution
29 Contribution of Angiotensin II to Supine Hypertension in Autonomic Failure Terminated NCT01292694 Phase 1 Losartan;Captopril;Placebo
30 Risk Prediction in Type II Diabetics With Ischemic Heart Disease Unknown status NCT01422057
31 Physiopathological Study of Autonomic Failure in Parkinson's Disease Completed NCT01748409 Not Applicable
32 Plasma Exchange for Autoimmune Autonomic Failure Completed NCT00359346
33 Autonomic Failure Patients for RNA Blood Sampling Completed NCT00742586
34 Norepinephrine Transporter Blockade as a Pathological Biomarker in Neurogenic Orthostatic Hypotension Completed NCT01316666
35 Nebivolol in the Supine Hypertension of Autonomic Failure Completed NCT01044693 Not Applicable Placebo;Nebivolol 5 mg;metoprolol tartrate 50 mg;Sildenafil25 mg
36 Nitric Oxide and the Autonomic Nervous System Completed NCT00178919 Not Applicable L-NMMA;Trimethaphan
37 Hypoglycemia Associated Autonomic Failure in Type 1 DM Completed NCT00592670 Not Applicable Fluoxetine;Placebo
38 Hypoglycemia Associated Autonomic Failure in Type 1 DM, Q2 Completed NCT00592332 Not Applicable Alprazolam
39 Abdominal Compression in Orthostatic Hypotension Completed NCT01223391 Not Applicable
40 Study of Idiopathic Edema Completed NCT01681927
41 Testing Glial Pathways to HAAF in Human Subjects Using Carbon 13 Magnetic Resonance Spectroscopy Completed NCT02690168
42 Posture Detection for Automated Abdominal Binder Completed NCT02280369
43 UTLight-transcranial Doppler Assessment of Regional Cerebral Autoregulation Completed NCT02178787
44 Risk Prediction in Aortic Stenosis Completed NCT01422044
45 Importance of Liver Innervation for the Osmopressor Response in Humans Completed NCT01237431 Not Applicable
46 Study of Norepinephrine Levels and Sympathetic Nervous System Activity Completed NCT00001329
47 Recurrent Hypoglycaemia in Type 1 Diabetes Completed NCT01337362 Not Applicable
48 Treatment of Supine Hypertension in Autonomic Failure (CPAP) Recruiting NCT03312556 Not Applicable Placebo
49 Overnight Trials With Heat Stress in Autonomic Failure Patients With Supine Hypertension Recruiting NCT03042988 Not Applicable
50 Local Heat Stress in Autonomic Failure Patients With Supine Hypertension Recruiting NCT02417415 Not Applicable

Search NIH Clinical Center for Pure Autonomic Failure

Genetic Tests for Pure Autonomic Failure

Genetic tests related to Pure Autonomic Failure:

# Genetic test Affiliating Genes
1 Orthostatic Hypotension 29

Anatomical Context for Pure Autonomic Failure

MalaCards organs/tissues related to Pure Autonomic Failure:

41
Heart, Brain, Spinal Cord, Liver, Skin, Testes, Cingulate Cortex

Publications for Pure Autonomic Failure

Articles related to Pure Autonomic Failure:

(show top 50) (show all 93)
# Title Authors Year
1
Does sympathetic dysfunction occur before denervation in pure autonomic failure? ( 29162745 )
2018
2
Sympathetic neurograms showing characteristics of both muscle and skin sympathetic nerve activity in a case with pure autonomic failure. ( 29313154 )
2018
3
Pure autonomic failure: Predictors of conversion to clinical CNS involvement. ( 28202694 )
2017
4
Pure autonomic failure without synucleinopathy. ( 28188385 )
2017
5
Very late-onset pure autonomic failure. ( 28436596 )
2017
6
Pure autonomic failure. ( 28717053 )
2017
7
Natural history of pure autonomic failure: A United States prospective cohort. ( 28093795 )
2017
8
Letter on "Natural history of pure autonomic failure: A United States prospective cohort". ( 28472851 )
2017
9
Reply to "Pure autonomic failure vs. Manifest CNS synucleinopathy: Relevance of stridor and autonomic biomarkers". ( 28472861 )
2017
10
Is pure autonomic failure a distinct nosologic entity? ( 28303413 )
2017
11
Pure autonomic failure without alpha-synuclein pathology: an evolving understanding of a heterogeneous disease. ( 28236005 )
2017
12
Is pure autonomic failure an early marker for Parkinson disease, dementia with Lewy bodies, and multiple system atrophy? And other updates on recent autonomic research. ( 28255741 )
2017
13
Managing neurogenic orthostatic hypotension in a patient presenting with pure autonomic failure who later developed Parkinson disease. ( 28699048 )
2017
14
Pure autonomic failure versus prodromal dysautonomia in Parkinsona89s disease: Insights from the bedside. ( 28164144 )
2017
15
Skin nerve misfolded I+-synuclein in pure autonomic failure and Parkinson disease. ( 26606657 )
2016
16
Pure Autonomic Failure. ( 27338613 )
2016
17
A case series of REM sleep behavior disorder in pure autonomic failure. ( 27757562 )
2016
18
Successful long-term ambulatory norepinephrine infusions in a patient with pure autonomic failure. ( 26138858 )
2015
19
Alpha-synuclein accumulation in a patient with Auerbach's plexus of pure autonomic failure. ( 25274243 )
2014
20
Skin sympathetic fiber I+-synuclein deposits: A potential biomarker for pure autonomic failure. ( 24379100 )
2014
21
Antemortem detection of colonic I+-synuclein pathology in a patient with pure autonomic failure. ( 25319021 )
2014
22
Incidence of cerebrovascular lesions in pure autonomic failure. ( 23706609 )
2013
23
Pure autonomic failure. ( 24095130 )
2013
24
Pure autonomic failure with cold induced sweating. ( 23511064 )
2013
25
Skin sympathetic fiber I+-synuclein deposits: a potential biomarker for pure autonomic failure. ( 23390175 )
2013
26
I+-Synuclein in the skin nerve of pure autonomic failure. ( 22186004 )
2011
27
Autonomic innervation in multiple system atrophy and pure autonomic failure. ( 20660924 )
2010
28
alpha-synuclein accumulation in skin nerve fibers revealed by skin biopsy in pure autonomic failure. ( 20157164 )
2010
29
Pure autonomic failure: a restricted Lewy body synucleinopathy or early Parkinson disease? ( 20157156 )
2010
30
Differences in skin sympathetic involvements between two chronic autonomic disorders: multiple system atrophy and pure autonomic failure. ( 18805037 )
2009
31
Reduced perfusion in the anterior cingulate cortex of patients with pure autonomic failure: an 123I-IMP SPECT study. ( 19684240 )
2009
32
Hyposmia in pure autonomic failure. ( 19433741 )
2009
33
Exercise-induced hypertension in pure autonomic failure. ( 19538201 )
2009
34
Olfactory dysfunction in pure autonomic failure: Implications for the pathogenesis of Lewy body diseases. ( 19201246 )
2009
35
Renal impairment of pure autonomic failure. ( 19738158 )
2009
36
Frequent nocturnal vocalization in pure autonomic failure. ( 18534130 )
2008
37
Effects of water drinking on cardiovascular responses to supine exercise and on orthostatic hypotension after exercise in pure autonomic failure. ( 18469030 )
2008
38
Central dopamine deficiency in pure autonomic failure. ( 18363034 )
2008
39
Pure autonomic failure followed by amyotrophy. ( 17339594 )
2007
40
REM sleep behavioral disorder in pure autonomic failure (PAF). ( 16505328 )
2006
41
Hemodynamic effects of clonidine in two contrasting models of autonomic failure: multiple system atrophy and pure autonomic failure. ( 16404729 )
2006
42
Chronic intestinal pseudo-obstruction as the initial feature of pure autonomic failure. ( 16705209 )
2006
43
Stress induced hypotension in pure autonomic failure. ( 16354738 )
2006
44
Cevimeline hydrochloride improved cholinergic dysfunction in a patient with pure autonomic failure. ( 15995794 )
2006
45
Reduced cardiac uptake and enhanced washout of 123I-MIBG in pure autonomic failure occurs conjointly with Parkinson's disease and dementia with Lewy bodies. ( 16818943 )
2006
46
Cognitive functioning in orthostatic hypotension due to pure autonomic failure. ( 16683070 )
2006
47
Skin vasomotor reflex responses in two contrasting groups of autonomic failure: multiple system atrophy and pure autonomic failure. ( 16845569 )
2006
48
Pure autonomic failure with altered dopamine transporter imaging. ( 16606777 )
2006
49
Cerebral vasomotor reactivity in Parkinson's disease, multiple system atrophy and pure autonomic failure. ( 16438986 )
2006
50
Cardiac and extracardiac sympathetic denervation in Parkinson's disease with orthostatic hypotension and in pure autonomic failure. ( 16269589 )
2005

Variations for Pure Autonomic Failure

Expression for Pure Autonomic Failure

Search GEO for disease gene expression data for Pure Autonomic Failure.

Pathways for Pure Autonomic Failure

Pathways related to Pure Autonomic Failure according to GeneCards Suite gene sharing:

(show all 20)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.6 DDC GNB3 MAOA MAOB NPPA SNCA
2
Show member pathways
12.87 ACHE DDC GNB3 MAOA MAOB TH
3 12.41 DBH DDC GNB3 SNCA TH
4
Show member pathways
12.1 ACE ACHE GNB3 REN
5 12.02 DDC GNB3 MAOA TH
6
Show member pathways
11.8 DDC MAOA MAOB TH
7 11.76 DDC GNB3 MAOA MAOB
8 11.65 DDC SNCA TH
9 11.63 DBH DDC MAOA TH
10
Show member pathways
11.43 DBH DDC MAOA MAOB TH
11
Show member pathways
11.27 DBH DDC TH
12
Show member pathways
11.19 ACHE DBH MAOA TH
13 11.14 MAOB SNCA TH
14 11.04 DDC MAOA
15
Show member pathways
11 ACHE MAOA
16
Show member pathways
10.74 MAOA MAOB
17
Show member pathways
10.67 MAOA MAOB
18 10.58 ACHE DDC TH
19 10.4 DDC MAOA MAOB TH
20
Show member pathways
10.36 ACHE DBH DDC MAOA TH

GO Terms for Pure Autonomic Failure

Cellular components related to Pure Autonomic Failure according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.56 ACE ACHE DBH EPO NPPA REN
2 synaptic vesicle GO:0008021 9.33 DDC SNCA TH
3 extracellular space GO:0005615 9.23 ACE ACHE DBH EPO NPPA REN

Biological processes related to Pure Autonomic Failure according to GeneCards Suite gene sharing:

(show all 28)
# Name GO ID Score Top Affiliating Genes
1 response to drug GO:0042493 9.86 MAOB REN SNCA TH
2 response to hypoxia GO:0001666 9.81 EPO NPPA TH
3 response to lipopolysaccharide GO:0032496 9.72 EPO MAOB REN SNCA TH
4 cellular response to drug GO:0035690 9.69 DDC REN TH
5 regulation of blood pressure GO:0008217 9.67 ACE GNB3 NPPA REN
6 response to electrical stimulus GO:0051602 9.63 EPO TH
7 response to amphetamine GO:0001975 9.63 DBH TH
8 positive regulation of endocytosis GO:0045807 9.62 NEDD4L SNCA
9 response to immobilization stress GO:0035902 9.62 REN TH
10 response to metal ion GO:0010038 9.61 NEDD4L TH
11 synaptic transmission, dopaminergic GO:0001963 9.61 SNCA TH
12 multicellular organism aging GO:0010259 9.59 DDC TH
13 response to corticosterone GO:0051412 9.58 MAOB TH
14 amyloid-beta metabolic process GO:0050435 9.58 ACE REN
15 neurotransmitter biosynthetic process GO:0042136 9.57 ACHE TH
16 angiotensin maturation GO:0002003 9.55 ACE REN
17 cellular response to alkaloid GO:0071312 9.54 DDC TH
18 response to salt stress GO:0009651 9.52 EPO TH
19 norepinephrine biosynthetic process GO:0042421 9.49 DBH TH
20 aminergic neurotransmitter loading into synaptic vesicle GO:0015842 9.48 DDC TH
21 response to pyrethroid GO:0046684 9.46 DDC TH
22 isoquinoline alkaloid metabolic process GO:0033076 9.43 DDC TH
23 neurotransmitter catabolic process GO:0042135 9.43 ACHE MAOA MAOB
24 phytoalexin metabolic process GO:0052314 9.4 DDC TH
25 dopamine biosynthetic process GO:0042416 9.33 DDC SNCA TH
26 dopamine catabolic process GO:0042420 9.13 DBH MAOA MAOB
27 catecholamine biosynthetic process GO:0042423 8.8 DBH DDC TH
28 oxidation-reduction process GO:0055114 10 DBH MAOA MAOB SNCA TH

Molecular functions related to Pure Autonomic Failure according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 oxidoreductase activity GO:0016491 9.35 DBH MAOA MAOB SNCA TH
2 hormone activity GO:0005179 9.33 EPO NPPA TTR
3 amino acid binding GO:0016597 9.32 DDC TH
4 ferrous iron binding GO:0008198 9.26 SNCA TH
5 primary amine oxidase activity GO:0008131 8.62 MAOA MAOB

Sources for Pure Autonomic Failure

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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