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TCEB1 - Humpath.com - Human pathology

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TCEB1

Tuesday 28 February 2017

Definition: Transcription elongation factor B polypeptide 1 is a protein that in humans is encoded by the TCEB1 gene.

This gene encodes the protein elongin C, which is a subunit of the transcription factor B (SIII) complex. The SIII complex is composed of elongins A/A2, B and C. It activates elongation by RNA polymerase II by suppressing transient pausing of the polymerase at many sites within transcription units.

Elongin A functions as the transcriptionally active component of the SIII complex, whereas elongins B and C are regulatory subunits.

Elongin A2 is specifically expressed in the testis, and capable of forming a stable complex with elongins B and C.

The von Hippel-Lindau tumor suppressor protein binds to elongins B and C, and thereby inhibits transcription elongation.

Pathology

- TCEB1-mutated renal cell carcinoma : TCEB1 mutations in renal cell carcinoma (RCC)

- SEC14L1 and TCEB1 genes are potential markers of progression. (23083832)

Open references

- TCEB1-mutated renal cell carcinoma: a distinct genomic and morphological subtype. Hakimi AA, Tickoo SK, Jacobsen A, Sarungbam J, Sfakianos JP, Sato Y, Morikawa T, Kume H, Fukayama M, Homma Y, Chen YB, Sankin AI, Mano R, Coleman JA, Russo P, Ogawa S, Sander C, Hsieh JJ, Reuter VE. Mod Pathol. 2015 Jun;28(6):845-53. doi : 10.1038/modpathol.2015.6 PMID: 25676555 Free