KURU, SUSCEPTIBILITY TO

General Information (adopted from Orphanet):

Synonyms, Signs:
Number of Symptoms 7
OrphanetNr:
OMIM Id: 245300
ICD-10:
UMLs:
MeSH:
MedDRA:
Snomed:

Prevalence, inheritance and age of onset:

Prevalence: No data available.
Inheritance:
Age of onset:

Disease classification (adopted from Orphanet):

Parent Diseases: No data available.

Symptom Information: Sort by abundance 

1
(HPO:0000496) Abnormality of eye movement 79 / 7739
2
(HPO:0001251) Ataxia 413 / 7739
3
(HPO:0001268) Mental deterioration 88 / 7739
4
(HPO:0002317) Unsteady gait 45 / 7739
5
(HPO:0002180) Neurodegeneration 31 / 7739
6
(OMIM) Unsteady stance and gait 1 / 7739
7
(OMIM) Increased brain astrocytes and neuronal degeneration with cytoplasmic vacuolization 1 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference

Additional Information:

Description: (OMIM) 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 ...
Clinical Description OMIM Collinge et al. (2006) identified 11 patients with kuru in Papua New Guinea who were all born before the cessation of cannibalism. The most likely incubation period in men ranged from 39 to 56 years. Most of the ...
Molecular genetics OMIM By PRNP genotyping of frozen blood samples from 92 patients with kuru, Cervenakova et al. (1998) found that homozygosity at codon 129 (M129V; 176640.0005), particularly for methionine, was associated with significantly earlier age at onset and a shorter ...
Population genetics OMIM Kuru is largely restricted to the Fore linguistic group of the Papua New Guinea Eastern Highlands and was transmitted during endocannibalistic feasts (Mead et al., 2003). Heterozygosity for a common polymorphism in the human prion protein gene confers ...