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Literature DB >> 62363

Characterization of heteropolymeric hexosaminidase A in human X mouse hybrid cells.

J Chern, E Beutler, W Kuhl, F Gilbert, W J Mellman, C M Croce.

Abstract

Expression of heteropolymeric hexosaminidase A activity is reported in a human X mouse hybrid cell line that contains an X/15 translocation chromosome but lacks human chromosome 5 and has no detectable human hexosaminidase B activity. (Hexosaminidase is beta-N-acetylglucosaminidase; EC 3.2.1.30; 2-acetamido-2-deoxy-beta-D-glucoside acetamidodeoxyglucohydrolase.) That the "hexosaminidase A" enzyme of these cells contains the human hexosaminidase-alpha subunit and not the human hexosaminidase-beta subunit is indicated by the cells' reactions to specific antisera prepared against the alpha subunit and against the beta subunit. Our results indicate that the "hexosaminidase A" activity in this hybrid cell line is the expression of a hybrid molecule composed of human hexosaminidase-alpha subunit and a mouse hexosaminidase subunit. These results are consistent with the hypothesis that the human hexosaminidase A enzyme is formed from alpha and beta subunits coded for by genes on chromosomes 15 and 5, respectively.

Entities: Gene Species

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Year: 1976 PMID： 62363 PMCID： PMC431173 DOI： 10.1073/pnas.73.10.3637

Source DB: PubMed Journal: Proc Natl Acad Sci U S A ISSN： 0027-8424 Impact factor: 11.205

28 in total

1. Subunit structure of human hexosaminidase verified: interconvertibility of hexosaminidase isozymes.

Authors: E Beutler; W Kuhl
Journal: Nature Date: 1975-11-20 Impact factor: 49.962

2. Assignment of the human gene for hexosaminidase B to chromosome 5.

Authors: H J Boedecker; W J Mellman; T A Tedesco; C M Croce
Journal: Exp Cell Res Date: 1975-07 Impact factor: 3.905

3. SELECTION OF HYBRIDS FROM MATINGS OF FIBROBLASTS IN VITRO AND THEIR PRESUMED RECOMBINANTS.

Authors: J W LITTLEFIELD
Journal: Science Date: 1964-08-14 Impact factor: 47.728

4. Assignment of the gene for cytoplasmic glutamic-oxaloacetic transaminase to the region q-24-qter of human chromosome 10.

Authors: C J Chern; W J Mellman; C M Croce
Journal: Somatic Cell Genet Date: 1976-03

5. Confirmation of the synteny of the human genes for mannose phosphate isomerase and pyruvate kinase and of their assignment to chromosome 15.

Authors: C J Chern; C M Croce
Journal: Cytogenet Cell Genet Date: 1975

6. Chromosome assignment of some human enzyme loci: mitochondrial malate dehydrogenase to 7, mannosephosphate isomerase and pyruvate kinase to 15 and probably, esterase D to 13.

Authors: V van Heyningen; M Bobrow; W F Bodmer; S E Gardiner; S Povey; D A Hopkinson
Journal: Ann Hum Genet Date: 1975-01 Impact factor: 1.670

7. Characterization of Hex S, the major residual beta hexosaminidase activity in type O Gm2 gangliosidosis (Sandhoff-Jatzkewitz disease).

Authors: J U Ikonne; M C Rattazzi; R J Desnick
Journal: Am J Hum Genet Date: 1975-09 Impact factor: 11.025

8. Assignment of the structural gene for human beta glucuronidase to chromosome 7 and tetrameric association of subunits in the enzyme molecule.

Authors: C J Chern; C M Croce
Journal: Am J Hum Genet Date: 1976-07 Impact factor: 11.025

5. A new immunochemical method for the quantitative measurement of specific gene products in man-rodent somatic cell hybrids.

Authors: P G de Groot; M N Hamers; A Westerveld; A W Schram; P Meera Khan; J M Tager
Journal: Hum Genet Date: 1978-11-16 Impact factor: 4.132

6. Detection of active heteropolymeric beta-glucuronidase in hybrids between mouse cells and human fibroblasts with beta-glucuronidase deficiency.

Authors: C J Chern
Journal: Proc Natl Acad Sci U S A Date: 1977-07 Impact factor: 11.205

6 in total

Characterization of heteropolymeric hexosaminidase A in human X mouse hybrid cells.

1. Subunit structure of human hexosaminidase verified: interconvertibility of hexosaminidase isozymes.

2. Assignment of the human gene for hexosaminidase B to chromosome 5.

3. SELECTION OF HYBRIDS FROM MATINGS OF FIBROBLASTS IN VITRO AND THEIR PRESUMED RECOMBINANTS.

4. Assignment of the gene for cytoplasmic glutamic-oxaloacetic transaminase to the region q-24-qter of human chromosome 10.

5. Confirmation of the synteny of the human genes for mannose phosphate isomerase and pyruvate kinase and of their assignment to chromosome 15.

6. Chromosome assignment of some human enzyme loci: mitochondrial malate dehydrogenase to 7, mannosephosphate isomerase and pyruvate kinase to 15 and probably, esterase D to 13.

7. Characterization of Hex S, the major residual beta hexosaminidase activity in type O Gm2 gangliosidosis (Sandhoff-Jatzkewitz disease).

8. Assignment of the structural gene for human beta glucuronidase to chromosome 7 and tetrameric association of subunits in the enzyme molecule.

9. Hexosaminidase-A and hexosaminidase-B: studies in Tay-Sachs' and Sandhoff's disease.

10. Hexosaminidase C in Tay-Sachs and Sandhoff disease.

Review 1. Biochemistry and genetics of gangliosidoses.

2. Mechanism of interrupted saccades in patients with late-onset Tay-Sachs disease.

Review 3. Glycosphingolipid hydrolases: properties and molecular genetics.

Review 4. The biochemical genetics of the hexosaminidase system in man.

5. A new immunochemical method for the quantitative measurement of specific gene products in man-rodent somatic cell hybrids.

6. Detection of active heteropolymeric beta-glucuronidase in hybrids between mouse cells and human fibroblasts with beta-glucuronidase deficiency.