ID

45675

Description

Principal Investigator: Helen Su, National Institute of Allergy and Infectious Diseases, Bethesda, MD, USA MeSH: Immunologic Deficiency Syndromes,RNA Virus Infections,Respiratory Tract Infections https://www.ncbi.nlm.nih.gov/projects/gap/cgi-bin/study.cgi?study_id=phs001235 We studied a child with life-threatening and recurrent respiratory tract infections, caused by multiple viruses including rhinovirus, influenza virus, and respiratory syncytial virus (RSV). We identified in her a homozygous missense mutation in IFIH1 that encodes MDA5 by Whole Exome Sequencing. MDA5-deficiency is a novel inborn error of innate immunity that results in impaired dsRNA-sensing, reduced IFN induction, and susceptibility to the common cold virus.

Link

dbGaP study = phs001235

Keywords

  1. 4/6/23 4/6/23 - Simon Heim
Copyright Holder

Helen Su, National Institute of Allergy and Infectious Diseases, Bethesda, MD, USA

Uploaded on

April 6, 2023

DOI

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License

Creative Commons BY 4.0

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dbGaP phs001235 Rhinovirus Susceptibility in Human MDA5 Deficiency

Eligibility Criteria

Inclusion and exclusion criteria
Description

Inclusion and exclusion criteria

Alias
UMLS CUI [1,1]
C1512693
UMLS CUI [1,2]
C0680251
Patient samples were chosen for inclusion in the project based on the following criteria:
Description

Patient samples were chosen for inclusion in the project based on the following criteria:

Data type

boolean

Alias
UMLS CUI [1,1]
C1512693
UMLS CUI [1,2]
C0243161
Families who had syndromic features were studied
Description

Families who had syndromic features were studied

Data type

boolean

Alias
UMLS CUI [1,1]
C0015576
UMLS CUI [1,2]
C5447321
Patients, parents and other family members are available
Description

Patients, parents and other family members are available

Data type

boolean

Alias
UMLS CUI [1,1]
C0470187
UMLS CUI [1,2]
C0030705
UMLS CUI [1,3]
C0030551
UMLS CUI [1,4]
C0086282
DNA was available for use in exome sequencing
Description

DNA was available for use in exome sequencing

Data type

boolean

Alias
UMLS CUI [1,1]
C0470187
UMLS CUI [1,2]
C0012854
UMLS CUI [1,3]
C5575842
Informed consent documentation
Description

Informed consent documentation

Data type

boolean

Alias
UMLS CUI [1,1]
C0021430

Similar models

Eligibility Criteria

Name
Type
Description | Question | Decode (Coded Value)
Data type
Alias
Item Group
Inclusion and exclusion criteria
C1512693 (UMLS CUI [1,1])
C0680251 (UMLS CUI [1,2])
Patient samples were chosen for inclusion in the project based on the following criteria:
Item
Patient samples were chosen for inclusion in the project based on the following criteria:
boolean
C1512693 (UMLS CUI [1,1])
C0243161 (UMLS CUI [1,2])
Families who had syndromic features were studied
Item
Families who had syndromic features were studied
boolean
C0015576 (UMLS CUI [1,1])
C5447321 (UMLS CUI [1,2])
Patients, parents and other family members are available
Item
Patients, parents and other family members are available
boolean
C0470187 (UMLS CUI [1,1])
C0030705 (UMLS CUI [1,2])
C0030551 (UMLS CUI [1,3])
C0086282 (UMLS CUI [1,4])
DNA was available for use in exome sequencing
Item
DNA was available for use in exome sequencing
boolean
C0470187 (UMLS CUI [1,1])
C0012854 (UMLS CUI [1,2])
C5575842 (UMLS CUI [1,3])
Informed consent documentation
Item
Informed consent documentation
boolean
C0021430 (UMLS CUI [1,1])

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