Abstract
A karyotype 46,XY,del(20)(q11 X 23q13 X 11) was found in a three year old boy with mental and growth retardation, low set ears, broad nasal bridge, and macrostomia. Adenosine deaminase (ADA) activity was reduced by about 50%, assigning the gene locus to the deleted segment. A review of the previously reported regional assignments suggests that the ADA gene is in the region of band 20q13 X 11.
MeSH Terms
Adenosine Deaminase/deficiency,genetics
Child, Preschool
Chromosome Deletion
Chromosome Mapping
Chromosomes, Human, Pair 20
Facial Bones/abnormalities
Growth Disorders/enzymology,genetics
Humans
Intellectual Disability/enzymology,genetics
Male
Nucleoside Deaminases/genetics
Skull/abnormalities
Chemicals
Nucleoside Deaminases
Adenosine Deaminase
Authors & Affiliations
5 authors, click to expand affiliations / ORCID
Petersen M B
Tranebjaerg L
Tommerup N
Nygaard P
Edwards H
References (5)
5 references, click to expand
-
Detection of the carrier state in combined immunodeficiency disease associated with adenosine deaminase deficiency.
J Clin Invest. 1974 Apr;53(4):1194-6
PMID: 4815083
-
Human adenosine deaminase and chromosome 20.
Experientia. 1978 Apr 15;34(4):531-2
PMID: 639957
-
[Partial monosomy 20q: a new syndrome. Regional assignment of the adenosine deaminase (ADA) locus on 20q132 (author's transl)].
Ann Genet. 1981;24(4):216-9
PMID: 6977300
-
Confirmation of the regional localization of the genes for human acid alpha-glucosidase (GAA) and adenosine deaminase (ADA) by somatic cell hybridization.
Ann Hum Genet. 1984 Jan;48(Pt 1):49-56
PMID: 6370091
-
Report of the Committee on the Genetic Constitution of Chromosomes 20, 21, and 22.
Cytogenet Cell Genet. 1985;40(1-4):268-95
PMID: 3864597