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ew syndromes in old genes; phenotypes caused by CREBBP and EP300 mutations but not resembling Rubinstein-Taybi syndrome

Completed
Conditions
at the end of exon 30 or beginning of exon 31)
intellectual disability due to mutations in CEEx30/31 (CREBBP or EP300
10083624
Registration Number
NL-OMON46364
Lead Sponsor
Academisch Medisch Centrum
Brief Summary

Not available

Detailed Description

Not available

Recruitment & Eligibility

Status
Completed
Sex
Not specified
Target Recruitment
36
Inclusion Criteria

1. Individuals with either a) a mutation in CEEx30/31, b) RSTS, c) a dup 16p13.3, d) a dup 22q13.2, and e) healthy age-, and sex-matched controls.
2. availability of stored fibroblasts or a planned surgical intervention for patient care reasons
3. Patients and/or parent(s)/caregiver(s)/legal representatives able to provide written permission

Exclusion Criteria

none

Study & Design

Study Type
Observational invasive
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
<p>We will compare the effects of the variants in CEEx30/31 with those of RSTS<br /><br>patients (CREBBP loss-of-function), patients with a duplication of CREBBP<br /><br>(CREBBP gain-of-function), and healthy controls on i) gene expression by<br /><br>RNA-Seq, ii) activity of DNA segments by ATAC-Seq, and iii) protein<br /><br>interactions with DNA using ChIP-seq. </p><br>
Secondary Outcome Measures
NameTimeMethod
<p>n.a.</p><br>
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