Proc Natl Acad Sci U S A. 1976 Oct;73(10):3637-40.

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

Chern J, Beutler E, Kuhl W, Gilbert F, Mellman WJ, Croce CM.

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.

PMID:: 62363; [PubMed - indexed for MEDLINE]
PMCID:: PMC431173

Free PMC Article

MeSH Terms, Substances

MeSH Terms

Substances

LinkOut - more resources

Full Text Sources

Medical

Supplemental Content

Save items

Related citations in PubMed

Tay-Sachs' and Sandhoff's diseases: the assignment of genes for hexosaminidase A and B to individual human chromosomes. [Proc Natl Acad Sci U S A. 1975]
Tay-Sachs' and Sandhoff's diseases: the assignment of genes for hexosaminidase A and B to individual human chromosomes.
Gilbert F, Kucherlapati R, Creagan RP, Murnane MJ, Darlington GJ, Ruddle FH. Proc Natl Acad Sci U S A. 1975 Jan; 72(1):263-7.
Interrelationship of hexosaminidases A and B: conformation of the common and the unique subunit theory. [Proc Natl Acad Sci U S A. 1976]
Interrelationship of hexosaminidases A and B: conformation of the common and the unique subunit theory.
Srivastava SK, Wiktorowicz JE, Awasthi YC. Proc Natl Acad Sci U S A. 1976 Aug; 73(8):2833-7.
Characterization of beta-D-N-acetylhexosaminidase isoenzymes in man-Chinese hamster somatic cell hybrids. [Am J Hum Genet. 1977]
Characterization of beta-D-N-acetylhexosaminidase isoenzymes in man-Chinese hamster somatic cell hybrids.
Hoeksema HL, Reuser AJ, Hoogeveen A, Westerveld A, Braidman I, Robinson D. Am J Hum Genet. 1977 Jan; 29(1):14-23.
Identification of domains in human beta-hexosaminidase that determine substrate specificity. [J Biol Chem. 1996]
Identification of domains in human beta-hexosaminidase that determine substrate specificity.
Pennybacker M, Liessem B, Moczall H, Tifft CJ, Sandhoff K, Proia RL. J Biol Chem. 1996 Jul 19; 271(29):17377-82.
Assignment of the structural genes for the alpha subunit of hexosaminidase A, mannosephosphate isomerase, and pyruvate kinase to the region q22-qter of human chromosome 15. [Somatic Cell Genet. 1977]
Assignment of the structural genes for the alpha subunit of hexosaminidase A, mannosephosphate isomerase, and pyruvate kinase to the region q22-qter of human chromosome 15.
Chern CJ, Kennett R, Engel E, Mellman WJ, Croce CM. Somatic Cell Genet. 1977 Nov; 3(6):553-60.

See reviews... See all...

Cited by 3 PubMed Central articles

Mechanism of interrupted saccades in patients with late-onset Tay-Sachs disease. [Prog Brain Res. 2008]
Mechanism of interrupted saccades in patients with late-onset Tay-Sachs disease.
Optican LM, Rucker JC, Keller EL, Leigh RJ. Prog Brain Res. 2008; 171:567-70.
Review The biochemical genetics of the hexosaminidase system in man. [Am J Hum Genet. 1979]
Review The biochemical genetics of the hexosaminidase system in man.
Beutler E. Am J Hum Genet. 1979 Mar; 31(2):95-105.
Detection of active heteropolymeric beta-glucuronidase in hybrids between mouse cells and human fibroblasts with beta-glucuronidase deficiency. [Proc Natl Acad Sci U S A. 1977]
Detection of active heteropolymeric beta-glucuronidase in hybrids between mouse cells and human fibroblasts with beta-glucuronidase deficiency.
Chern CJ. Proc Natl Acad Sci U S A. 1977 Jul; 74(7):2948-52.

Related information

Related Citations
Calculated set of PubMed citations closely related to the selected article(s) retrieved using a word weight algorithm. Related articles are displayed in ranked order from most to least relevant, with the “linked from” citation displayed first.
Gene (OMIM)
Gene records associated with Online Mendelian Inheritance in Man (OMIM) records that cite the current articles in their reference lists.
Nucleotide (Weighted)
Nucleotide records associated with the current articles through the Gene database. These are the related sequences on the Gene record that are added manually by NCBI.
OMIM (calculated)
Online Mendelian Inheritance in Man (OMIM) records that include the current articles as references in the light bulb links within or in the citations at the end of the OMIM record. The references available through the light bulb link are collected using the PubMed related articles algorithm to identify records with similar terminology to the OMIM record.
OMIM (cited)
Online Mendelian Inheritance in Man (OMIM) records that include the current articles as reference cited at the end of the OMIM record.
Protein (Weighted)
Protein records associated with the current articles through related Gene database records. These are the related sequences on the Gene record that are added manually by NCBI.
References for this PMC Article
Citation referenced in PubMed article. Only valid for PubMed citations that are also in PMC.
Free in PMC
Free full text articles in PMC
Cited in PMC
Full-text articles in the PubMed Central Database that cite the current articles.

Recent activity

Clear Turn Off Turn On

Characterization of heteropolymeric hexosaminidase A in human X mouse hybrid cel...
Characterization of heteropolymeric hexosaminidase A in human X mouse hybrid cells.
Proc Natl Acad Sci U S A. 1976 Oct ;73(10):3637-40.

PubMed
The DNA synthetic response of normal and abnormal human lymphocytes to mevalonic...
The DNA synthetic response of normal and abnormal human lymphocytes to mevalonic acid: the role of granulocytes as a helper population.
J Allergy Clin Immunol. 1984 Sep ;74(3 Pt 1):280-90.

PubMed
Heparan sulfate and dermatan sulfate inhibit the generation of thrombin activity...
Heparan sulfate and dermatan sulfate inhibit the generation of thrombin activity in plasma by complementary pathways.
Blood. 1984 Sep ;64(3):742-7.

PubMed
Human dihydrofolate reductase gene organization. Extensive conservation of the G...
Human dihydrofolate reductase gene organization. Extensive conservation of the G + C-rich 5' non-coding sequence and strong intron size divergence from homologous mammalian genes.
J Mol Biol. 1984 Jun 25 ;176(2):169-87.

PubMed
Investigations on the inheritance of nemaline myopathy.
Investigations on the inheritance of nemaline myopathy.
Arch Neurol. 1978 Feb ;35(2):72-7.

PubMed

Your browsing activity is empty.

Activity recording is turned off.

Turn recording back on

PubMed

Result Filters

Display Settings:

Send to: