Literature DB >> 8949721

Analysis of 21 Stargardt's disease families confirms a major locus on chromosome 1p with evidence for non-allelic heterogeneity in a minority of cases.

B H Weber1, S Sander, C Kopp, D Walker, A Eckstein, B Wissinger, E Zrenner, T Grimm.   

Abstract

BACKGROUND: Autosomal recessive Stargardt's disease is a macular degeneration characterised by a juvenile onset and a rapidly progressive course resulting in an atrophic macular area typically surrounded by yellowish retinal flecks.
METHOD: The disease locus has previously been assigned to markers from chromosome 1p21-p13 by genetic linkage analysis in eight multiplex Stargardt's disease families.
RESULTS: In an extended analysis, the assignment to chromosome 1p was confirmed in the majority of the 21 families with Stargardt's disease who were studied. In addition, a series of recombinant chromosomes further narrowed the Stargardt's disease region to an approximately 3 cM interval between markers at D1S424 and D1S497.
CONCLUSION: Multipoint linkage analysis most probably excludes this locus in three of these families suggesting non-allelic heterogeneity with at least one additional minor Stargardt's disease locus.

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Year:  1996        PMID: 8949721      PMCID: PMC505593          DOI: 10.1136/bjo.80.8.745

Source DB:  PubMed          Journal:  Br J Ophthalmol        ISSN: 0007-1161            Impact factor:   4.638


  18 in total

1.  Clinical and genetic heterogeneity in retinitis pigmentosa.

Authors:  J Kaplan; D Bonneau; J Frézal; A Munnich; J L Dufier
Journal:  Hum Genet       Date:  1990-10       Impact factor: 4.132

2.  Dominant inheritance of Stargardt's disease.

Authors:  P P Bither; L A Berns
Journal:  J Am Optom Assoc       Date:  1988-02

3.  Infantile type of so-called neuronal ceroid-lipofuscinosis. 1. A clinical study of 15 patients.

Authors:  P Santavuori; M Haltia; J Rapola; C Raitta
Journal:  J Neurol Sci       Date:  1973-03       Impact factor: 3.181

4.  Mapping the human amylase gene cluster on the proximal short arm of chromosome 1 using a highly informative (CA)n repeat.

Authors:  N C Dracopoli; M H Meisler
Journal:  Genomics       Date:  1990-05       Impact factor: 5.736

5.  Exclusion of the cone-specific alpha-subunit of the transducin gene in Stargardt's disease.

Authors:  S Gerber; J M Rozet; D Bonneau; E Souied; J Weissenbach; J Frezal; A Munnich; J Kaplan
Journal:  Hum Genet       Date:  1995-04       Impact factor: 4.132

6.  Long-term follow-up of dominant macular dystrophy with flecks (Stargardt).

Authors:  A M Mansour
Journal:  Ophthalmologica       Date:  1992       Impact factor: 3.250

7.  Multilocus linkage analysis in humans: detection of linkage and estimation of recombination.

Authors:  G M Lathrop; J M Lalouel; C Julier; J Ott
Journal:  Am J Hum Genet       Date:  1985-05       Impact factor: 11.025

8.  Dominantly inherited macular dystrophy with flecks (Stargardt).

Authors:  G W Cibis; M Morey; D J Harris
Journal:  Arch Ophthalmol       Date:  1980-10

Review 9.  Variability in the clinical and pathological findings in the neuronal ceroid lipofuscinoses: review of data and observations.

Authors:  K E Wisneiwski; E Kida; O F Patxot; F Connell
Journal:  Am J Med Genet       Date:  1992-02-15

10.  Mutations in the palmitoyl protein thioesterase gene causing infantile neuronal ceroid lipofuscinosis.

Authors:  J Vesa; E Hellsten; L A Verkruyse; L A Camp; J Rapola; P Santavuori; S L Hofmann; L Peltonen
Journal:  Nature       Date:  1995-08-17       Impact factor: 49.962

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  2 in total

1.  The 2588G-->C mutation in the ABCR gene is a mild frequent founder mutation in the Western European population and allows the classification of ABCR mutations in patients with Stargardt disease.

Authors:  A Maugeri; M A van Driel; D J van de Pol; B J Klevering; F J van Haren; N Tijmes; A A Bergen; K Rohrschneider; A Blankenagel; A J Pinckers; N Dahl; H G Brunner; A F Deutman; C B Hoyng; F P Cremers
Journal:  Am J Hum Genet       Date:  1999-04       Impact factor: 11.025

2.  Genotype/Phenotype analysis of a photoreceptor-specific ATP-binding cassette transporter gene, ABCR, in Stargardt disease.

Authors:  R A Lewis; N F Shroyer; N Singh; R Allikmets; A Hutchinson; Y Li; J R Lupski; M Leppert; M Dean
Journal:  Am J Hum Genet       Date:  1999-02       Impact factor: 11.025

  2 in total

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