Literature DB >> 6490007

A gene regulating the time dependence of alpha-L-fucosidase concentration is closely linked with the structural gene in man.

B Tümmler, M Duthie, M Buchwald, J R Riordan.   

Abstract

The dependence of the specific activities of human alpha-L-fucosidase and four other lysosomal glycosidases on cell growth was determined in long-term lymphoid cell lines in which the polymorphic forms of alpha-L-fucosidase were identified by isoelectric focusing. In cultured lymphoblasts of Fu-1 phenotype the alpha-L-fucosidase synthesis during cell growth was found to be proportional to the general amount of protein. However, in cells derived from individuals possessing at least one FUCA*2 allele, the amount of enzyme increased progressively during the log-phase of growth, reaching a maximum at the transition to the stationary phase. The maximum increase of enzyme concentration was observed in the mid-log phase of exponential growth. The results may be explained by the existence of a regulatory locus FUCT which maps in close proximity to the structural gene. The FUCT locus which controls the amount of enzyme in the cell appears to be specific for alpha-L-fucosidase.

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Year:  1984        PMID: 6490007     DOI: 10.1007/bf00291398

Source DB:  PubMed          Journal:  Hum Genet        ISSN: 0340-6717            Impact factor:   4.132


  20 in total

1.  Polymorphism of human alpha fucosidase.

Authors:  B M Turner; V S Turner; N G Beratis; K Hirschhorn
Journal:  Am J Hum Genet       Date:  1975-09       Impact factor: 11.025

Review 2.  Acid hydrolases as models of genetic control.

Authors:  K Paigen
Journal:  Annu Rev Genet       Date:  1979       Impact factor: 16.830

3.  Genetics of murine liver and kidney arysulfatase b.

Authors:  W L Daniel
Journal:  Genetics       Date:  1976-03-25       Impact factor: 4.562

4.  Assignment of the gene locus for human alpha-L-fucosidase to chromosome 1 by analysis of somatic cell hybrids.

Authors:  B M Turner; M Smith; V S Turner; R S Kucherlapati; F H Ruddle; K Hirschhorn
Journal:  Somatic Cell Genet       Date:  1978-01

5.  Isolation and characterization of the plasma membranes of cultured lymphoblasts from patients with cystic fibrosis and normal individuals.

Authors:  T Maler; J R Riordan
Journal:  Biochim Biophys Acta       Date:  1980-05-08

6.  alpha-L-Fucosidase is quantitatively reduced in cultured lymphoblasts from patients with cystic fibrosis.

Authors:  T Maler; M Duthie; N Alon; J R Riordan
Journal:  J Biol Chem       Date:  1981-02-10       Impact factor: 5.157

7.  Genetic control of heat sensitivity and activity level of murine arylsulfatase B.

Authors:  W L Daniel
Journal:  Biochem Genet       Date:  1976-12       Impact factor: 1.890

8.  Genetic determination of the beta-galactosidase developmental program in mouse liver.

Authors:  K Paigen; M Meisler; J Felton; V Chapman
Journal:  Cell       Date:  1976-12       Impact factor: 41.582

9.  N-Acetyl-beta-glucosaminidases in human spleen.

Authors:  D Robinson; J L Stirling
Journal:  Biochem J       Date:  1968-04       Impact factor: 3.857

10.  Gene transfer by means of cell fusion. II. The mapping of 8 loci on human chromosome 1 by statistical analysis of gene assortment in somatic cell hybrids.

Authors:  S J Goss; H Harris
Journal:  J Cell Sci       Date:  1977-06       Impact factor: 5.285

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  2 in total

1.  Variation in alpha-L-fucosidase properties among 28 inbred mouse strains: six strains have high enzyme activity and heat-stabile enzyme with a variant pH-activity curve; twenty-two strains have low activity and heat-labile enzyme.

Authors:  W G Johnson; J L Hong
Journal:  Biochem Genet       Date:  1986-06       Impact factor: 1.890

2.  Biosynthesis, processing, and extracellular release of alpha-L-fucosidase in lymphoid cell lines of different genetic origins.

Authors:  R A DiCioccio; K S Brown
Journal:  Biochem Genet       Date:  1988-06       Impact factor: 1.890

  2 in total

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