Entry - *600786 - ELONGIN A; ELOA - OMIM
 
* 600786

ELONGIN A; ELOA


Alternative titles; symbols

TRANSCRIPTION ELONGATION FACTOR B, POLYPEPTIDE 3; TCEB3
TCEB3A
ELONGIN A1
ELONGIN, 110-KD SUBUNIT


HGNC Approved Gene Symbol: ELOA

Cytogenetic location: 1p36.11     Genomic coordinates (GRCh38): 1:23,743,471-23,762,059 (from NCBI)


TEXT

Description

Eukaryotic transcription and mRNA synthesis are complex biochemical processes controlled in part by the concerted action of a set of general transcription factors that regulate the activity of RNA polymerase II (see 180660) at both the initiation and elongation stages of transcription. Aso et al. (1995) noted that several general initiation factors, including TFIIA (see 600520), TFIIB (189963), TFIID (see 313650), TFIIE (see 189962), and TFIIH (see 189972), and several accessory proteins (e.g., TAFs; see 600475), have been identified in eukaryotic cells and found to promote selective binding of RNA polymerase II to promoters and to support a basal level of transcription.

In addition to the general initiation factors, 3 general elongation factors, SII (601425), TFIIF, and elongin (SIII), have been defined biochemically in eukaryotes and shown to increase the overall rate at which RNA polymerase II transcribes duplex DNA. TFIIF from higher eukaryotes is a heterodimer composed of RAP74, a subunit of approximately 70 kD (189968) and RAP30 (189969), a subunit of approximately 30 kD. Elongin (also referred to as SIII) is a heterotrimer composed of A, B (ELOB; 600787), and C (ELOC; 600788) subunits of 110, 18, and 15 kD, respectively. The complex activates elongation of mammalian RNA polymerase II by suppressing transient pausing of the polymerase at many sites within transcription units. (The genes encoding elongins A, B, and C have also been symbolized TCEB3, TCEB2, and TCEB1, respectively.)


Cloning and Expression

Aso et al. (1995) isolated the rat elongin A gene. Aso et al. (1996) described a full-length cDNA encoding the human homolog of rat Eloa. The predicted 722-amino acid protein shares 84% homology with the rat protein. Comparison of the open reading frames of the human cDNA with that of the previously characterized rat cDNA indicated that they are 84% conserved in nucleotide sequence.


Gene Function

Aso et al. (1995) isolated the rat elongin A gene, expressed the gene, and reconstituted the elongin complex with recombinant subunits. They showed that elongin A functions as the transcriptionally active component of the complex and elongin B and C as regulatory subunits. Whereas elongin C assembled with elongin A to form an AC complex with increased specific activity, elongin B, a member of the ubiquitin-homology gene family, appeared to serve a chaperone-like function, facilitating assembly and enhancing stability of the elongin complex.

Duan et al. (1995) and Kibel et al. (1995) reported results suggesting that the tumor suppression activity of the von Hippel-Lindau tumor suppressor gene product (VHL; 608537) is a function of its ability to bind to TCEB2 and TCEB1 and inhibit transcription elongation.


Mapping

By fluorescence in situ hybridization, Aso et al. (1995) mapped the TCEB3 gene to 1p36.1. They pointed out that this region has been shown to have deletions in several forms of malignancy.


REFERENCES

  1. Aso, T., Haque, D., Fukudome, K., Brower, C. S., Conaway, J. W., Conaway, R. C. A human cDNA encoding the 110-kDa A subunit of RNA polymerase II transcription factor elongin. Gene 168: 277-278, 1996. [PubMed: 8654961, related citations] [Full Text]

  2. Aso, T., Lane, W. S., Conaway, J. W., Conaway, R. C. Elongin (SIII): a multisubunit regulator of elongation by RNA polymerase II. Science 269: 1439-1443, 1995. [PubMed: 7660129, related citations] [Full Text]

  3. Aso, T., Mokady, N., Haque, D., Conaway, R. C., Conaway, J. W. Assignment of a human gene encoding the 110-kDa subunit of general transcription factor elongin (SIII) to chromosome 1p36.1. Genomics 30: 393-394, 1995. [PubMed: 8586449, related citations]

  4. Duan, D. R., Pause, A., Burgess, W. H., Aso, T., Chen, D. Y. T., Garrett, K. P., Conaway, R. C., Conaway, J. W., Linehan, W. M., Klausner, R. D. Inhibition of transcription elongation by the VHL tumor suppressor protein. Science 269: 1402-1406, 1995. [PubMed: 7660122, related citations] [Full Text]

  5. Kibel, A., Iliopoulos, O., DeCaprio, J. A., Kaelin, W. G., Jr. Binding of the von Hippel-Lindau tumor suppressor protein to elongin B and C. Science 269: 1444-1446, 1995. [PubMed: 7660130, related citations] [Full Text]


Contributors:
Alan F. Scott - updated : 1/14/1996
Creation Date:
Victor A. McKusick : 10/6/1995
carol : 11/03/2017
mgross : 08/08/2005
ckniffin : 3/23/2004
alopez : 5/21/1999
carol : 12/15/1998
mark : 3/28/1997
terry : 9/17/1996
terry : 9/17/1996
mark : 5/9/1996
terry : 5/2/1996
joanna : 4/11/1996
mark : 1/14/1996
mark : 10/6/1995

* 600786

ELONGIN A; ELOA


Alternative titles; symbols

TRANSCRIPTION ELONGATION FACTOR B, POLYPEPTIDE 3; TCEB3
TCEB3A
ELONGIN A1
ELONGIN, 110-KD SUBUNIT


HGNC Approved Gene Symbol: ELOA

Cytogenetic location: 1p36.11     Genomic coordinates (GRCh38): 1:23,743,471-23,762,059 (from NCBI)


TEXT

Description

Eukaryotic transcription and mRNA synthesis are complex biochemical processes controlled in part by the concerted action of a set of general transcription factors that regulate the activity of RNA polymerase II (see 180660) at both the initiation and elongation stages of transcription. Aso et al. (1995) noted that several general initiation factors, including TFIIA (see 600520), TFIIB (189963), TFIID (see 313650), TFIIE (see 189962), and TFIIH (see 189972), and several accessory proteins (e.g., TAFs; see 600475), have been identified in eukaryotic cells and found to promote selective binding of RNA polymerase II to promoters and to support a basal level of transcription.

In addition to the general initiation factors, 3 general elongation factors, SII (601425), TFIIF, and elongin (SIII), have been defined biochemically in eukaryotes and shown to increase the overall rate at which RNA polymerase II transcribes duplex DNA. TFIIF from higher eukaryotes is a heterodimer composed of RAP74, a subunit of approximately 70 kD (189968) and RAP30 (189969), a subunit of approximately 30 kD. Elongin (also referred to as SIII) is a heterotrimer composed of A, B (ELOB; 600787), and C (ELOC; 600788) subunits of 110, 18, and 15 kD, respectively. The complex activates elongation of mammalian RNA polymerase II by suppressing transient pausing of the polymerase at many sites within transcription units. (The genes encoding elongins A, B, and C have also been symbolized TCEB3, TCEB2, and TCEB1, respectively.)


Cloning and Expression

Aso et al. (1995) isolated the rat elongin A gene. Aso et al. (1996) described a full-length cDNA encoding the human homolog of rat Eloa. The predicted 722-amino acid protein shares 84% homology with the rat protein. Comparison of the open reading frames of the human cDNA with that of the previously characterized rat cDNA indicated that they are 84% conserved in nucleotide sequence.


Gene Function

Aso et al. (1995) isolated the rat elongin A gene, expressed the gene, and reconstituted the elongin complex with recombinant subunits. They showed that elongin A functions as the transcriptionally active component of the complex and elongin B and C as regulatory subunits. Whereas elongin C assembled with elongin A to form an AC complex with increased specific activity, elongin B, a member of the ubiquitin-homology gene family, appeared to serve a chaperone-like function, facilitating assembly and enhancing stability of the elongin complex.

Duan et al. (1995) and Kibel et al. (1995) reported results suggesting that the tumor suppression activity of the von Hippel-Lindau tumor suppressor gene product (VHL; 608537) is a function of its ability to bind to TCEB2 and TCEB1 and inhibit transcription elongation.


Mapping

By fluorescence in situ hybridization, Aso et al. (1995) mapped the TCEB3 gene to 1p36.1. They pointed out that this region has been shown to have deletions in several forms of malignancy.


REFERENCES

  1. Aso, T., Haque, D., Fukudome, K., Brower, C. S., Conaway, J. W., Conaway, R. C. A human cDNA encoding the 110-kDa A subunit of RNA polymerase II transcription factor elongin. Gene 168: 277-278, 1996. [PubMed: 8654961] [Full Text: https://doi.org/10.1016/0378-1119(95)00750-4]

  2. Aso, T., Lane, W. S., Conaway, J. W., Conaway, R. C. Elongin (SIII): a multisubunit regulator of elongation by RNA polymerase II. Science 269: 1439-1443, 1995. [PubMed: 7660129] [Full Text: https://doi.org/10.1126/science.7660129]

  3. Aso, T., Mokady, N., Haque, D., Conaway, R. C., Conaway, J. W. Assignment of a human gene encoding the 110-kDa subunit of general transcription factor elongin (SIII) to chromosome 1p36.1. Genomics 30: 393-394, 1995. [PubMed: 8586449]

  4. Duan, D. R., Pause, A., Burgess, W. H., Aso, T., Chen, D. Y. T., Garrett, K. P., Conaway, R. C., Conaway, J. W., Linehan, W. M., Klausner, R. D. Inhibition of transcription elongation by the VHL tumor suppressor protein. Science 269: 1402-1406, 1995. [PubMed: 7660122] [Full Text: https://doi.org/10.1126/science.7660122]

  5. Kibel, A., Iliopoulos, O., DeCaprio, J. A., Kaelin, W. G., Jr. Binding of the von Hippel-Lindau tumor suppressor protein to elongin B and C. Science 269: 1444-1446, 1995. [PubMed: 7660130] [Full Text: https://doi.org/10.1126/science.7660130]


Contributors:
Alan F. Scott - updated : 1/14/1996

Creation Date:
Victor A. McKusick : 10/6/1995

Edit History:
carol : 11/03/2017
mgross : 08/08/2005
ckniffin : 3/23/2004
alopez : 5/21/1999
carol : 12/15/1998
mark : 3/28/1997
terry : 9/17/1996
terry : 9/17/1996
mark : 5/9/1996
terry : 5/2/1996
joanna : 4/11/1996
mark : 1/14/1996
mark : 10/6/1995