Submission Details

Submitter:

Classification:
Definitive
GENCC:100001
Gene:
Disease:
TELO2-related intellectual disability-neurodevelopmental disorder
Mode Of Inheritance:
Autosomal recessive
Evaluated Date:
06/01/2022
Evidence/Notes:

TELO2 was first reported in relation to autosomal recesssive syndromic intellectual disability and neurodevelopmental disorder in 2016 (You et al., PMID:27132593). TELO2-related intellectual disability-neurodevelopmental disorder (You-Hoover-Fong syndrome) is characterized by delayed global development, microcephaly, abnormal balance, and a movement disorder. At least 11 variants (including missense, nonsense, frameshift, exonic deletion) have been reported (PMID: 28944240, 33307281, 32940098). Evidence supporting this gene-disease relationship includes case-level and experimental data. In summary, there is definitive evidence supporting the relationship between TELO2 and TELO2-related intellectual disability-neurodevelopmental disorder. This classification was approved by the ClinGen Intellectual Disability and Autism Gene Curation Expert Panel on June 1, 2022 (SOP Version 9).

PubMed IDs:
19344873 20810650 27132593 28944240 32940098 33307281
Public Report:
Assertion Criteria:
Submitter Submitted Date:
12/05/2025

The GenCC data are available free of restriction under a CC0 1.0 Universal (CC0 1.0) Public Domain Dedication. The GenCC requests that you give attribution to GenCC and the contributing sources whenever possible and appropriate. The accepted Flagship manuscript is now available from Genetics in Medicine (https://www.gimjournal.org/article/S1098-3600(22)00746-8/fulltext).

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