Abstract
The localisation of the gene for Hunter syndrome (MPS II) has been studied in 11 families using 12 polymorphic DNA markers, one on the short arm and the remaining 11 located at various points on the long arm of the X chromosome. Lod scores for seven probes were uniformly negative for all values of theta; positive scores at values of theta = 0.10 or more were obtained for the five probes located most distally on the long arm (52A, F9C, DX13, St14-1, F8C). Current data suggest the most likely order of the loci to be: 52A, F9C, Hunter, DX13, St14-1, F8C-qter; the Hunter locus may thus be close to that for the fragile site at Xq27.
MeSH Terms
Chromosome Banding
Chromosome Mapping
DNA/genetics
Female
Genetic Linkage
Genetic Markers
Humans
Male
Mucopolysaccharidosis II/genetics
Pedigree
Polymorphism, Restriction Fragment Length
X Chromosome
Chemicals
Genetic Markers
DNA
Authors & Affiliations
7 authors, click to expand affiliations / ORCID
Upadhyaya M
Sarfarazi M
Bamforth J S
Thomas N S
Oberle I
Young I
Harper P S
References (21)
21 references, click to expand
-
Carrier detection in Hunter syndrome.
Am J Med Genet. 1983 Sep;16(1):61-9
PMID: 6227239
-
Strategies for multilocus linkage analysis in humans.
Proc Natl Acad Sci U S A. 1984 Jun;81(11):3443-6
PMID: 6587361
-
Estimation of the recombination fraction in human pedigrees: efficient computation of the likelihood for human linkage studies.
Am J Hum Genet. 1974 Sep;26(5):588-97
PMID: 4422075
-
Analysis of human Y-chromosome-specific reiterated DNA in chromosome variants.
Proc Natl Acad Sci U S A. 1977 Mar;74(3):1245-9
PMID: 265567
-
Cloning of a representative genomic library of the human X chromosome after sorting by flow cytometry.
Nature. 1981 Oct 1;293(5831):374-6
PMID: 6456416
-
"A technique for radiolabeling DNA restriction endonuclease fragments to high specific activity". Addendum.
Anal Biochem. 1984 Feb;137(1):266-7
PMID: 6329026
-
A clinically useful DNA probe closely linked to haemophilia A.
Lancet. 1984 Jul 7;2(8393):6-8
PMID: 6145983
-
Genetic mapping and diagnosis of haemophilia A achieved through a BclI polymorphism in the factor VIII gene.
Nature. 1985 Apr 25-May 1;314(6013):738-40
PMID: 2986011
-
Carrier detection in haemophilia B using two further intragenic restriction fragment length polymorphisms.
Nucleic Acids Res. 1984 Dec 11;12(23):8861-72
PMID: 6096810
-
The telomeric region of the human X chromosome long arm: presence of a highly polymorphic DNA marker and analysis of recombination frequency.
Proc Natl Acad Sci U S A. 1985 May;82(9):2824-8
PMID: 2986139
-
Single-copy sequence hybridizes to polymorphic and homologous loci on human X and Y chromosomes.
Proc Natl Acad Sci U S A. 1982 Sep;79(17):5352-6
PMID: 6291041
-
Duchenne muscular dystrophy (DMD) in a female with an X/autosome translocation: further evidence that the DMD locus is at Xp21.
Am J Hum Genet. 1981 Jul;33(4):513-8
PMID: 7258185
-
Localization by in situ hybridization of the coagulation factor IX gene and of two polymorphic DNA probes with respect to the fragile X site.
Hum Genet. 1985;69(4):327-31
PMID: 2985491
-
The linkage relation of the loci for the Xm serum system and the X-linked form of Hurler's syndrome (Hunter's syndrome).
Am J Hum Genet. 1968 Jul;20(4):398-401
PMID: 4969416
-
Hunter's disease in a girl: association with X:5 chromosomal translocation disrupting the Hunter gene.
Arch Dis Child. 1983 Nov;58(11):911-5
PMID: 6418082
-
Characterisation and use of an intragenic polymorphic marker for detection of carriers of haemophilia B (factor IX deficiency).
Lancet. 1984 Feb 4;1(8371):239-41
PMID: 6142992
-
First trimester prenatal diagnosis of adrenoleukodystrophy by determination of very long chain fatty acid levels and by linkage analysis to a DNA probe.
Hum Genet. 1985;69(3):272-4
PMID: 3856557
-
Translocation (X;6) in a female with Duchenne muscular dystrophy: implications for the localisation of the DMD locus.
J Med Genet. 1981 Dec;18(6):442-7
PMID: 7334502
-
Polyclonal antibodies against iduronate 2-sulphate sulphatase from human urine.
Biochim Biophys Acta. 1984 Oct 16;801(3):365-71
PMID: 6386054
-
Isolation of probes detecting restriction fragment length polymorphisms from X chromosome-specific libraries: potential use for diagnosis of Duchenne muscular dystrophy.
Hum Genet. 1985;70(2):148-56
PMID: 2989153
-
The defect in the Hunter syndrome: deficiency of sulfoiduronate sulfatase.
Proc Natl Acad Sci U S A. 1973 Jul;70(7):2134-8
PMID: 4269173