FAME4 YEATS2

Disease ID
FAME4
Gene ID
YEATS2
Updated
Feb 7, 2025
v2.3.1

Disease

Name
Familial adult myoclonic epilepsy 4
Inheritance
Autosomal dominant
Description
Cortical tremor, seizures with generalised motor (tonic-clonic) onset1 .
Prevalence
Age of OnsetYears10  33
Age of Onset Details
Typical: 20-252 ; Range: 10-333 .

Locus

Details
Novel insertion of ~1000 repeats observed to cause disease3 .
Mechanism
GoF?
RNA toxicity hypothesized3 .
Year
20193
Location in Gene
Intron 1
Gene Strand

Alleles

Ref. Motif
TTTTA
Pathogenic (ref.)
TTTCA
Pathogenic (gene)
ATTTC
Unknown (ref.)
TTTTT, TGTTA
Unknown (gene)
TTTTT, ATGTT
PathogenicUnits1,000  1,000

gnomAD

Pathogenic Genotype (%)Admixed AmericanAfrican/African AmericanAmishAshkenazi JewishEuropean (non Finnish)FinnishMiddle EasternSouth AsianEast AsianOthers01234567YEATS2

Pathogenic Genotype (%): % of individuals predicted to be affected based on their genotype

References

Direct supporting references for info on this page.

1
The Lancet. Neurology · 2024-07-01
pmid:38876750
2
European journal of human genetics : EJHG · 2012-06-20
pmid:22713812
3
Brain : a journal of neurology · 2019-11-01
pmid:31539032

Additional Literature

Additional literature related to this locus.

Raw PubMed search results
(All PubMed results returned by searching for this gene, tandem repeats, and disease, in medline format)

Progress in neurobiology · 2023-12-20
pmid:38128822