MCID: KR002
MIFTS: 38

Kuru

Categories: Genetic diseases, Rare diseases, Neuronal diseases

Aliases & Classifications for Kuru

MalaCards integrated aliases for Kuru:

Name: Kuru 57 12 76 53 54 59 75 75 55 44 15 73
Kuru, Susceptibility to 57 29 13 40
Kuru Encephalopathy 12

Characteristics:

Orphanet epidemiological data:

59
kuru
Inheritance: Multigenic/multifactorial,Not applicable; Prevalence: <1/1000000 (Worldwide); Age of onset: All ages; Age of death: any age;

OMIM:

57
Inheritance:
'slow virus' etiology with ? genetic factors


Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

OMIM 57 245300
Disease Ontology 12 DOID:648
ICD10 33 A81.81
ICD9CM 35 046.0
MeSH 44 D007729
SNOMED-CT 68 192684001 86188000
Orphanet 59 ORPHA454745
UMLS via Orphanet 74 C0022802
ICD10 via Orphanet 34 A81.8
MedGen 42 C1855588
UMLS 73 C0022802

Summaries for Kuru

NINDS : 54 Kuru is a rare and fatal brain disorder that occurred at epidemic levels during the 1950s-60s among the Fore people in the highlands of New Guinea. The disease was the result of the practice of ritualistic cannibalism among the Fore, in which relatives prepared and consumed the tissues (including brain) of deceased family members. Brain tissue from individuals with kuru was highly infectious, and the disease was transmitted either through eating or by contact with open sores or wounds. Government discouragement of the practice of cannibalism led to a continuing decline in the disease, which has now mostly disappeared. Kuru belongs to a class of infectious diseases called transmissible spongiform encephalopathies (TSEs), also known as prion diseases. The hallmark of a TSE disease is misshapen protein molecules that clump together and accumulate in brain tissue. Scientists believe that misshapen prion proteins have the ability to change their shape and cause other proteins of the same type to also change shape. Other TSEs include Creutzfeldt-Jakob disease and fatal familial insomnia in humans, bovine spongiform encephalopathy in cattle (also known as mad cow disease), scrapie in sheep and goats, and chronic wasting disease in deer and elk.

MalaCards based summary : Kuru, also known as kuru, susceptibility to, is related to fatal familial insomnia and prion disease. An important gene associated with Kuru is PRNP (Prion Protein). The drugs Carvedilol and Hyaluronic acid have been mentioned in the context of this disorder. Affiliated tissues include brain, heart and bone, and related phenotypes are abnormality of eye movement and ataxia

OMIM : 57 Kuru, a fatal neurodegenerative condition, is a human prion disease that primarily affected the Fore linguistic group of the Eastern Highlands of Papua New Guinea. Kuru was transmitted by the practice of consuming dead relatives as a mark of respect and mourning ('transumption'). The incidence has fallen dramatically since the cessation of cannibalism in the 1950s (summary by Wadsworth et al., 2008). (245300)

UniProtKB/Swiss-Prot : 75 Kuru: Kuru is transmitted during ritualistic cannibalism, among natives of the New Guinea highlands. Patients exhibit various movement disorders like cerebellar abnormalities, rigidity of the limbs, and clonus. Emotional lability is present, and dementia is conspicuously absent. Death usually occurs from 3 to 12 month after onset.

Wikipedia : 76 Kuru is a very rare, incurable neurodegenerative disorder that was formerly common among the Fore people... more...

Related Diseases for Kuru

Diseases related to Kuru via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 13)
# Related Disease Score Top Affiliating Genes
1 fatal familial insomnia 28.2 PRNP RASA2 ZBTB38
2 prion disease 11.4
3 genetic prion diseases 11.4
4 creutzfeldt-jakob disease 11.3
5 gerstmann-straussler disease 10.9
6 scrapie 10.1
7 encephalopathy 10.1
8 alzheimer disease 10.0
9 cerebritis 10.0
10 prostatitis 9.8
11 cerebellar degeneration 9.8
12 subacute cerebellar degeneration 9.8
13 chronic wasting disease 9.1 ERC1 PRNP

Graphical network of the top 20 diseases related to Kuru:



Diseases related to Kuru

Symptoms & Phenotypes for Kuru

Symptoms via clinical synopsis from OMIM:

57
Neuro:
unsteady stance and gait
ataxia
abnormal extraocular movement
mental deterioration

Lab:
increased brain astrocytes and neuronal degeneration with cytoplasmic vacuolization


Clinical features from OMIM:

245300

Human phenotypes related to Kuru:

32
# Description HPO Frequency HPO Source Accession
1 abnormality of eye movement 32 HP:0000496
2 ataxia 32 HP:0001251
3 mental deterioration 32 HP:0001268
4 neurodegeneration 32 HP:0002180
5 unsteady gait 32 HP:0002317

Drugs & Therapeutics for Kuru

Drugs for Kuru (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 21)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Carvedilol Approved, Investigational Phase 4 72956-09-3 2585
2
Hyaluronic acid Approved, Vet_approved Phase 4 9004-61-9 53477741
3 Adrenergic Agents Phase 4
4 Adrenergic alpha-1 Receptor Antagonists Phase 4
5 Adrenergic alpha-Antagonists Phase 4
6 Adrenergic Antagonists Phase 4
7 Adrenergic beta-Antagonists Phase 4
8 Antihypertensive Agents Phase 4,Phase 3,Phase 2
9 Neurotransmitter Agents Phase 4
10 Vasodilator Agents Phase 4
11 Mitogens Phase 4
12 Adjuvants, Immunologic Phase 4
13 Anti-Inflammatory Agents Phase 4
14 Protective Agents Phase 4
15 Viscosupplements Phase 4
16
Treprostinil Approved, Investigational Phase 3,Phase 2 81846-19-7 54786 6918140
17 Pharmaceutical Solutions Phase 2, Phase 3,Not Applicable
18 Photosensitizing Agents Not Applicable
19 Tolonium Chloride Not Applicable
20 Calamus Nutraceutical Not Applicable
21 Bifidobacterium Nutraceutical Early Phase 1

Interventional clinical trials:

(show all 12)
# Name Status NCT ID Phase Drugs
1 The Preventive Efficacy of Carvedilol on Cardiac Dysfunction in Duchenne Muscular Dystrophy Unknown status NCT00606775 Phase 4 Carvedilol
2 Treatment of Intrabony Periodontal Defects With Enamel Matrix Derivatives and Autogenous Bone Graft Completed NCT02218515 Phase 4
3 Effect of Topically-Applied Hyaluronic-Acid on Palatal Epithelial Wound Closure Completed NCT02534415 Phase 4 Periodontal dressing material;0.2% Hyaluronic acid gel;0.8% Hyaluronic acid gel
4 Extended Access Program to Assess Long-term Safety of Bardoxolone Methyl in Patients With Pulmonary Hypertension RANGER Recruiting NCT03068130 Phase 3 Bardoxolone methyl
5 Study of Oral Treprostinil in Subjects With Pulmonary Hypertension (PH) Associated With Heart Failure With Preserved Ejection Fraction (HFpEF) Recruiting NCT03043651 Phase 3 Oral Treprostinil
6 Oral Treprostinil in Subjects With Pulmonary Hypertension (PH) Associated With Heart Failure With Preserved Ejection Fraction (HFpEF) Recruiting NCT03037580 Phase 3 Oral treprostinil;Placebo
7 Bardoxolone Methyl in Patients With Connective Tissue Disease-associated Pulmonary Arterial Hypertension - CATALYST Recruiting NCT02657356 Phase 3 Placebo capsules;Bardoxolone methyl capsules
8 Safety and Efficacy of Inhaled Treprostinil in Adult PH With ILD Including CPFE Recruiting NCT02630316 Phase 2, Phase 3 Inhaled Treprostinil;Placebo
9 Effects Of Adjunctive Photodynamic Therapy To Non-Surgical Periodontal Treatment In Patients With Generalized Aggressive Periodontitis Completed NCT03412331 Not Applicable
10 Probiotic Yoghurt Effect on Experimental Gingivitis Completed NCT02546206 Early Phase 1
11 Time Difference Between Onsets of Lateral And Medial Hamstring Muscles During Gait in Patients With Patellofemoral Pain. Completed NCT03392688 Not Applicable
12 Is There a Relationship Between Hyperlipidemia and Periodontitis? Completed NCT02826720

Search NIH Clinical Center for Kuru

Cochrane evidence based reviews: kuru

Genetic Tests for Kuru

Genetic tests related to Kuru:

# Genetic test Affiliating Genes
1 Kuru, Susceptibility to 29 PRNP

Anatomical Context for Kuru

MalaCards organs/tissues related to Kuru:

41
Brain, Heart, Bone, Eye, Prostate, Testes, Cortex

Publications for Kuru

Articles related to Kuru:

(show top 50) (show all 56)
# Title Authors Year
1
Cultural factors that affected the spatial and temporal epidemiology of kuru. ( 28280581 )
2017
2
An autopsied case of MV2KA +A C-type sporadic Creutzfeldt-Jakob disease presenting with widespread cerebral cortical involvement and Kuru plaques. ( 28568896 )
2017
3
Experimental and numerical study of drill bit drop tests on Kuru granite. ( 27956511 )
2017
4
Cannibalism, Kuru, and Mad Cows: Prion Disease As a &amp;quot;Choose-Your-Own-Experiment&amp;quot; Case Study to Simulate Scientific Inquiry in Large Lectures. ( 26788803 )
2016
5
Correction: Cannibalism, Kuru, and Mad Cows: Prion Disease As a &amp;quot;Choose-Your-Own-Experiment&amp;quot; Case Study to Simulate Scientific Inquiry in Large Lectures. ( 26991940 )
2016
6
An Enduring Shell Artefact Tradition from Timor-Leste: Oliva Bead Production from the Pleistocene to Late Holocene at Jerimalai, Lene Hara, and Matja Kuru 1 and 2. ( 27537696 )
2016
7
Atypical sporadic CJD-MM phenotype with white matter kuru plaques associated with intranuclear inclusion body and argyrophilic grain disease. ( 25783686 )
2015
8
Kuru: a journey back in time from papua new Guinea to the neanderthals' extinction. ( 25437203 )
2013
9
Re: Phantom study of a novel stereotactic prostate biopsy system integrating preinterventional magnetic resonance imaging and live ultrasonography fusion (from: Kuru TH, Roethke M, Popeneclu V, et al. J Endourol 2012;26:807-813.). ( 22835032 )
2013
10
Atypical neuropathological sCJD-MM phenotype with abundant white matter Kuru-type plaques sparing the cerebellar cortex. ( 22862687 )
2013
11
Epidemiological mechanisms of genetic resistance to kuru. ( 23740487 )
2013
12
Kuru: the first prion disease. ( 22411241 )
2012
13
Human prion diseases: from kuru to variant creutzfeldt-jakob disease. ( 23225013 )
2012
14
Kuru: genes, cannibals and neuropathology. ( 22249461 )
2012
15
Replication and spread of CJD, kuru and scrapie agents in vivo and in cell culture. ( 21527829 )
2011
16
Cannibalism, kuru and anthropology. ( 19618336 )
2009
17
Kuru and D. Carleton Gajdusek: a close encounter. ( 19618335 )
2009
18
A novel protective prion protein variant that colocalizes with kuru exposure. ( 19923577 )
2009
19
Ultrastructural study of florid plaques in variant Creutzfeldt-Jakob disease: a comparison with amyloid plaques in kuru, sporadic Creutzfeldt-Jakob disease and Gerstmann-StrAoussler-Scheinker disease. ( 18513219 )
2009
20
The kuru infectious agent is a unique geographic isolate distinct from Creutzfeldt-Jakob disease and scrapie agents. ( 19633190 )
2009
21
Review. Lessons of kuru research: background to recent studies with some personal reflections. ( 18849283 )
2008
22
Review. The origin of the prion agent of kuru: molecular and biological strain typing. ( 18849291 )
2008
23
Review. The neuropathology of kuru and variant Creutzfeldt-Jakob disease. ( 18849282 )
2008
24
Genetic susceptibility, evolution and the kuru epidemic. ( 18849290 )
2008
25
Kuru prions and sporadic Creutzfeldt-Jakob disease prions have equivalent transmission properties in transgenic and wild-type mice. ( 18316717 )
2008
26
Mortuary rites of the South Fore and kuru. ( 18849288 )
2008
27
Central and peripheral pathology of kuru: pathological analysis of a recent case and comparison with other forms of human prion disease. ( 18849292 )
2008
28
Subependymal plaques in scrapie-affected hamster brains--why are they so different from compact kuru plaques? ( 18368625 )
2008
29
A clinical study of kuru patients with long incubation periods at the end of the epidemic in Papua New Guinea. ( 18849289 )
2008
30
Kuru likened to scrapie: the story remembered. ( 18849258 )
2008
31
Review. Understanding kuru: the contribution of anthropology and medicine. ( 18849287 )
2008
32
Review. Kuru and its contribution to medicine. ( 18849284 )
2008
33
Review. The epidemiology of kuru: monitoring the epidemic from its peak to its end. ( 18849286 )
2008
34
Familial Creutzfeldt-Jakob disease with an R208H-129V haplotype and Kuru plaques. ( 16533975 )
2006
35
Kuru in the 21st century--an acquired human prion disease with very long incubation periods. ( 16798390 )
2006
36
Autopsy case of Creutzfeldt-Jakob disease with Met/Val heterozygosity at codon 129 and type 1 protease-resistant prion protein presenting some florid-type plaques and many Kuru plaques in the cerebellum. ( 16961067 )
2006
37
Genetic studies in relation to kuru: an overview. ( 15354868 )
2004
38
Acquired prion disease: iatrogenic CJD, variant CJD, kuru. ( 14522863 )
2003
39
Increased susceptibility to Kuru of carriers of the PRNP 129 methionine/methionine genotype. ( 11120925 )
2001
40
Gerstmann-StrAoussler-Scheinker syndrome,fatal familial insomnia, and kuru: a review of these less common human transmissible spongiform encephalopathies. ( 11535002 )
2001
41
Phenotype-genotype studies in kuru: implications for new variant Creutzfeldt-Jakob disease. ( 9789072 )
1998
42
Is the neuropathology of new variant Creutzfeldt-Jakob disease and kuru similar? ( 9250194 )
1997
43
Pathology and immunocytochemistry of a kuru brain. ( 9034563 )
1997
44
Proliferating cell nuclear antigen immunohistochemistry in astrocytes in experimental Creutzfeldt-Jakob disease and in human kuru, Creutzfeldt-Jakob disease and Gerstmann-StrAoussler-Scheinker syndrome. ( 7583684 )
1995
45
Apolipoprotein E immunoreactivity in cerebral amyloid deposits and neurofibrillary tangles in Alzheimer's disease and kuru plaque amyloid in Creutzfeldt-Jakob disease. ( 2029618 )
1991
46
N-terminal sequence of prion protein is also integrated into kuru plaques in patients with Gerstmann-StrAoussler syndrome. ( 1677605 )
1991
47
A comparative immunohistochemical study of Kuru and senile plaques with a special reference to glial reactions at various stages of amyloid plaque formation. ( 1679596 )
1991
48
A prion protein missense variant is integrated in kuru plaque cores in patients with Gerstmann-StrAoussler syndrome. ( 1671530 )
1991
49
Alzheimer's amyloid precursor protein-positive degenerative neurites exist even within kuru plaques not specific to Alzheimer's disease. ( 1684265 )
1991
50
Creutzfeldt-Jakob disease patients with congophilic kuru plaques have the missense variant prion protein common to Gerstmann-StrAoussler syndrome. ( 2180366 )
1990

Variations for Kuru

Expression for Kuru

Search GEO for disease gene expression data for Kuru.

Pathways for Kuru

GO Terms for Kuru

Cellular components related to Kuru according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 postsynaptic density GO:0014069 8.62 CPEB1 PRNP

Sources for Kuru

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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